30
Facial Pain from Various Sources- Diagnoses and Differential Diagnoses Course # 4 Nelson Hendler, MD, MS, Former Assistant Professor of Neurosurgery Johns Hopkins University School of Medicine Former Associate Professor of Physiology University of Maryland School of Dental Surgery Past president-American Academy of Pain Management www.DiagnoseMyPain.com

Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

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This lecture covers the most common sources of facial pain, including trigeminal neuralgia, TMJ, Sjogren's, Eagles syndrome, glossopharyngeal neuralgia and other..This is based on a lecture on facial pain, given at University of Maryland School of Dental Surgery

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Page 1: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Facial Pain from Various Sources-Diagnoses and Differential

Diagnoses

Course 4

Nelson Hendler MD MS

Former Assistant Professor of Neurosurgery

Johns Hopkins University School of Medicine

Former Associate Professor of Physiology

University of Maryland School of Dental Surgery

Past president-American Academy of Pain Management

wwwDiagnoseMyPaincom

Sensory Nerve Chart

for nerves which

cause pain to the

face Not only do

many of the 12 cranial

nerve (nerve which

come directly off of

the brain) have

painful conditions but

the nerve roots (nerve

which come off of the

spinal cord) also can

cause face pain The

C2-3 root may refer

pain to the angle of

the jaw or to an area

behind the eye The

occipital nerve may

give pain like a stripe

up the back of the

head

What is temporomandibular (TMJ) joint syndrome

bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw

bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone

bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

What is temporomandibular (TMJ) joint syndrome

bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull Irritation of these nerves produces pain in the jaw cheek andor temple

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw

(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 2: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Sensory Nerve Chart

for nerves which

cause pain to the

face Not only do

many of the 12 cranial

nerve (nerve which

come directly off of

the brain) have

painful conditions but

the nerve roots (nerve

which come off of the

spinal cord) also can

cause face pain The

C2-3 root may refer

pain to the angle of

the jaw or to an area

behind the eye The

occipital nerve may

give pain like a stripe

up the back of the

head

What is temporomandibular (TMJ) joint syndrome

bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw

bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone

bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

What is temporomandibular (TMJ) joint syndrome

bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull Irritation of these nerves produces pain in the jaw cheek andor temple

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw

(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 3: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is temporomandibular (TMJ) joint syndrome

bull Damage to the ligaments andor disc of the joint between the skull and the head of the jaw

bull The temporomandibular (TMJ) joint is a very unusual joint It is formed by the head of the condoyle of the jaw (mandible) inserting into a recess in the skull right beneath the temporal bone

bull The jaw bone is held to the skull by this joint and the ligaments that hold the jaw to the skull are the strong lateral temporomandibular ligaments and two weaker medial ligaments(Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

What is temporomandibular (TMJ) joint syndrome

bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull Irritation of these nerves produces pain in the jaw cheek andor temple

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw

(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 4: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is temporomandibular (TMJ) joint syndrome

bull The nerves that supply the joint are the auriculotemporal and massetric branches of the third division of the trigeminal nerve (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull Irritation of these nerves produces pain in the jaw cheek andor temple

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw

(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 5: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull Typical symptoms are bull facial pain in the cheek bull in the area of the joint bull in the jaw line bull ringing in the ear bull dizziness bull Pain in the temple and bull clicks in the jaw on opening or closing the jaw

(Gelb H TMJ Syndrome The tell-tale click Behavioral Medicine March 1978)

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 6: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you diagnosis temporomandibular (TMJ) joint syndrome

bull TMJ is often diagnosed as something else such as Menierrersquossyndrome or trigeminal neuralgia (CommentaryThe Great Imposter Diseases of the Temporomandibular (TMJ) joint JAMA Vol 235 No 22 May 31 1976)

bull Sometimes MRIs with the jaws open and shut or even better cine MRIs (MRI movies) will reveal a displaced meniscus between the jaw and the skull

bull Injection of frac14 cc of 05 bupivicaine in the thesternocleidomastoid muscle may give temporary relief thereby confirming the diagnosis (Travell J Temporomandibular Joint Pain Referred from Muscles of the Head and Neck Journal of Prosthetic Dentistry Volume 10 No 4 1960 pp 745 ndash 763 and Travell JG Guest Editorial A Conjoint Attack on the

Causes of Craniomandibular Pain Journal of CraniomandibularPractice March ndash May 1984 2109 ndash 110)

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 7: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you treat temporomandibular(TMJ) joint syndrome

bull Muscles relaxants and anti-inflammatory drugs such as iboprophen or naproxen are helpful (Guralnick W Kaban L and Merrill R Temporomandibular joint afflictions New England Journal of Medicine Vol 299 No 3 pp 120-129 July 20 1978)

bull The use of a Kil-Brux night guard may help which is a bite guard which fits over teeth on one side and with a smooth side on the other side so the opposing teeth can slide over the smooth surface

bull The dentist should never grind down the teeth which alters the bite and repositions the jaw

bull Only in rare instances is surgery recommended to repair the disc or ligaments and rarely does it improve the condition

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 8: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Temporal Bone

JAW

BONE

SKULL

Normal TMJ

anatomy The

condyle is the part

of the jaw bone

which inserts into

the recess in the

skull Ligaments

hold the jaw bone

into the recess or

fossa (another name

for recess) The

fossa is an

indentation in the

skull bone which

accepts the condyle

of the jaw bone

Think of ligaments

like stretched rubber

bands holding the

condyle into the

fossa

The disc is like a

cushion and a shock

absorber The disc is

sort of like a water

filled balloon which

allows the condyle to

roll around in the

fossa while being

held in place by

ligaments The jaw

can slide forward or

back side to side or

pivot-a 3 way joint

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 9: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

In TMJ either the

disc or ligaments are

torn and this allow

the condyle to move

more than it should

Sometime the disc

moves too and gets

trapped between the

front of the fossa and

the condyle when

the jaw is opened

This causes the click

heard with TMJ

which is sort of like

snapping your

fingers

Condyle

Disc

Fossa

ligaments

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 10: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is trigeminal neuralgia bull There are two main etiologies to the pain of trigeminal

neuralgia bull In both types the pain can be constant or intermittent and

follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull One etiology is a congenital compression of the trigeminal nerve by the middle cerebral artery in the skull This was well researched by Peter Janetta MD and his team at Uni of Pttisburgh

bull (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvasculardecompression for trigeminal neuralgia in elderly patients J Neuro Neurosurg2008 Apr108(4)689-91)

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 11: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

SENSORY DISTRIBUTION

OF THE TRIGEMINAL

NERVE V IS FOR ROMAN

NUMERAL FIVE THERE

ARE THREE SENSORY

BRANCHES V1 or the

ophthalmic branch V2 or

the maxillary branch AND

V3 or the mandibular

branch Notice all the

sensory sensation is in the

jaw cheek and forehead

nose and eye

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 12: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is trigeminal neuralgia

bull A second form of trigeminal neuralgia is a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull Interestingly the pain of trigeminal neuralgia from both sources is clinically the same

bull This is an example of ldquoconvergencerdquo which is a single clinical manifestation due to several etiologies

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 13: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you diagnose trigeminal neuralgia

bull The pain of both types of trigeminal neuralgia can be constant or intermittent and follows the path of the sensory branches of the trigeminal nerve into the forehead along the cheek bone or in the jaw line or any combination of the three (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990)

bull The pain is sensitive to light touch and may occur in burst of pain rather than being constant This occurs in both types of trigeminal neuralgia

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 14: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you diagnose trigeminal neuralgia

bull In order to differentiate between the two forms of trigeminal neuralgia an MRI of the brain with vascular enhancement is useful

bull If there is no vessel compression it is highly likely the cause of the pain is post-herpetic

bull In addition to an MRI with vascular enhancement an MRI of the brain with thin-sections (2 mm slices) through the trigeminal nerve and an MRA (Magnet resonance angiography) may be helpful in differentiating the post-viral etiology from the compression of the nerve by the blood vessel

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 15: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is the treatment of trigeminal neuralgia

bull Since there are two main etiologies to the pain of trigeminal neuralgia there are two types of treatment

bull If the etiology is compression of the trigeminal nerve by the middle cerebral artery in the skull then the treatment is intra-cranial decompression of the nerve by inserting a sponge between the artery and the nerve (Sekula RF Marchan EM Fletcher LH Casey KF Jannetta PJ Microvascular decompression for trigeminal neuralgia in elderly patients J NeuroNeurosurg2008 Apr108(4)689-91)

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 16: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is the treatment of trigeminal neuralgia

bull If the etiology is due to a herpetic-like viral irritation of the trigeminal nerve (Empting L Hendler N Kolodny L Kraus H Tips on hard to manage pain syndromes Patient Care page 26-46 April 30 1990) then the treatment is the use of medications

bull Some people respond to low dose anti-depressants but the most useful medication is an anti-convulsant such as gaba-pentin (Neurontin) or Tegretol

bull Anti-viral drug such as acyclovir will also help

bull Rarely radio-frequency lesions of the retro-gausserian ganglion can be employed if all else fails

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 17: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is Eagles syndrome

bull Eagles Syndrome is characterized by an a specific orofacial pain secondary to calcification of the stylohyoid ligament or elongated styoid process In about 4 of general population an elongated styloid process occurs while only about 4 of these patients are symptomatic The stylo-hyoid bone is considered abnormal if it is greater than 25 mm in length (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 18: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

3D-CT of a very long

stylo-hyoid

bonehellipmuch longer

than 25 mm Follow

the numbers 123

and 4 on both sides of

the 3D-CT and the is

the stylo-hyoid bone

which is about 39 mm

in this 3D-CT The

bone marked H is the

hyoid bone In this

3D-CT the stylo-hyoid

bone is so long that is

spontaneously broke

between 2 and 3 but it

is still symptomatic

and causes pain with

swollowing Removal

of the bone from the

break to 4 on the 3D-

CT will relieve

symptoms

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 19: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What are the symptoms of Eagles syndrome and how do you diagnosis itbull The patient has a sensation of a foreign body localized at

the tonsillar fossa associated with a dull intermittent painbull Pain is usually worse with swallowingbull A bony projection is usually palpable with bimanual trans-

oral explorationbull Basically you put your finger in the mouth of the patient

down their throat and push towards the outside where the stylohyoid bone is If this reproduces the pain this confirms the diagnosis

bull A lateral radiograph and a computed tomography scan of head and neck will show an elongated styloid process of at least 25 mm (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G Salvinelli FAtypical chronic head and neck pain dont forget Eagles syndrome Eur Rev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 20: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

How do you treat Eagles syndromebull Medical treatment represents the first choice

bull Use non-steroidal anti- inflammatory drugs

bull If this is not effective then surgical resection of the styloid process may help (cutting off the tip)

bull In the case of persistence or ingravescence of the complaint surgery may be the only option

bull Symptoms may overlap with glossopharyngealneuritis (presented later)

bull (Casale M Rinaldi V Quattrocchi C Bressi F Vincenzi B Santini D Tonini G

Salvinelli F Atypical chronic head and neck pain dont forget Eagles syndrome EurRev Med Pharmacol Sci 2008 Mar-Apr12(2)131-3)

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 21: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What is Sjogrenrsquos Syndrome bull Sjogren syndrome is an autoimmune disorder in

which the glands that produce tears and saliva are destroyed causing dry mouth and dry eyes

bull Symptoms may include itching eyes feeling that something is in the eye difficulty swallowing loss of sense of taste thick saliva mouth sores many cavities in the teeth and pain in the face usually in the trigeminal or facial nerve

bull (Kruszka P OBrian RJ Diagnosis and management of Sjogren syndrome Am Fam Physician 200979465-470)

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 22: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

What are symptoms of Sjogrenrsquos Syndromebull Can be mistaken for trigeminal neuralgia

bull When there is unexplained pain in the face take a careful history Ask about dry mouth itchy eyes lots of cavities trouble swallowing SICCA complex (1)

bull Trigeminal sensory neuropathy can occur with Sjogrenrsquos and may be characterized by progressive sensory complaints on the face They could start on one side and subsequently become bilateral (2)

bull (1) Wu AJ Optimizing dry mouth treatment for individuals with Sjoumlgrens syndrome Rheum Dis Clin North Am 2008 Nov34(4)1001-1010

bull (2) Steven Mandel MD Clinical Professor of Neurology Jefferson Medical College)

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 23: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Laboratory Tests to Diagnose Sjogrenrsquos

bull SSA and SSB ANA sed rate rheumatoid factor (RF) Schirmer test corneal fluorescein staining conjunctival lissamine green staining tear-film breakup time and lip biopsy are useful methods of diagnosing Sjogrenrsquos but not always reliable

bull Yang Z Liang Y Zhong R Is identification of anti-SSA andor -SSB antibodies necessary in serum samples referred for antinuclear antibodies testing J Clin Lab Anal 2012 Nov26(6)447-51 Br J Ophthalmol 2012 Dec96(12)1498-503 doi with aqueous-deficient dry eye

bull Liew MS Zhang M Kim E Akpek EK Prevalence and predictors of Sjogrens syndrome in a prospective cohort of patients with aqueous-deficient dry eye Br J Ophthalmol 2012 Dec96(12)1498-503

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 24: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Treatment of Sjogrenrsquosbull Conventional immunosuppressive therapies

such as corticosteroids have been used in some patients with variable benefit

bull Current therapy for dryness is principally symptomatic

bull The greatest experience in pSS is with rituximab an anti-CD20 (expressed on a subset of B-cells) monoclonal antibody

(Bowman S Barone F Biologic treatments in Sjoumlgrens syndrome Presse Med 2012 Sep41(9 Pt 2)e495-509)

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 25: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Symptoms of Glossopharyngeal Neuritis

bull Glossopharyngeal neuralgia is classically described as ldquostabbingrdquo or ldquoelectrical shock-likerdquo in nature Episodes are typically brief lasting a couple of seconds up to a few minutes and can be quite debilitating They can occur in the tongue throat ear and tonsils on one side of the head but in rare cases may be bilateral Painful attacks may occur spontaneously but are more often associated with a specific stimulus such as chewing speaking coughing swallowing or laughing The attacks can occur frequently throughout the day or once every couple of weeks and they can also disturb sleep

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 26: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Causes of Glossopharyngeal Neuralgiabull Neuralgia is typically caused by compression of the

glossopharyngeal nerve

bull Compression of the nerve by an adjacent artery andor vein can occur near the brainstem

bull Compression of the nerve more distally in the neck by an elongated styloid process (a bone in the neck) can cause this pain syndrome as well and this is called Eagle syndrome

bull Glossopharyngeal neuralgia can be caused by a tumor or infection in the region of ninth cranial nerve or by multiple sclerosis

bull httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 27: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Differential diagnosis of Glossopharyngeal Neuritis (IX)

bull The diagnosis is made on clinical grounds alone

bull no specific test can be done to prove the diagnosis in any given patient Episodic ear andor throat pain often triggered by touching the palate or tonsil is strongly suggestive of the disorder

bull High resolution MRI or CT imaging of the brainstem may reveal vascular compression tumors or demyelinating lesions involving the ninth cranial nerve

bull High resolution CT scanning of the neck can reveal an elongated styloid process suggestive of Eagle syndrome

bull Pain that distinctly involves the ear rear 13 of the tongue andor throat is the classic pain distribution seen in glossopharyngeal neuralgia (ninth cranial nerve or IX N)

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia

Page 28: Facial pain due to TMJ, trigminal neuralgia, Sjogren syndrome, Eagles etc

Treatment of Glossopharyngeal Neuritisbull Most treatment for Glossopharyngeal Neuralgia is

pharmacological Anticonvulsant medications including oxcarbazepine carbamazepine gabapentin and pregabalin Some antidepressants can also be helpful either alone or in combination with the seizure medications If medications fail surgical intervention is indicated

bull The first-choice treatment microvascular decompression (MVD) as it has the highest initial and long-term success rates Patients unable to tolerate such an operation may consider one of the many less invasive options including Gamma Knife radiosurgery

bull Eagles syndrome TMJ and Trigeminal neuralgia can co-exist with glossopharyngeal neuritis and mask the diagnosis or be mistaken for it

httpwwwcolumbianeurosurgeryorgconditionsglossopharyngeal-neuralgia