9/4/2019

1

COMMON SYMPTOMS OF UNCOMMON CEREBROVASCULAR DISORDERS: SOMETIMES IT REALLY

IS A ZEBRA.

Kim Page, NPUniversity of Rochester Medical Center

Rochester, NY

CEREBROVASCULAR DISORDERS

• Carotid and vertebral artery injuries

• Basilar artery disease

• Arteriovenous Fistulas

• Venous sinus thrombosis

• Cerebral small vessel diseases

• Vasculitis

• Central Retinal Artery Occlusion

CV TRAUMA: INJURIES TO THE CERVICAL ARTERIES

• Rare but associated with mortality rates 20-40%; permanent neurologic impairment 40-80%

• Symptoms frequently develop in a delayed fashion—important to have high index of suspicion based on mechanism of injury

• Biggest risk is thrombotic occlusion

1

2

3

9/4/2019

2

CAROTID & VERTEBRAL ARTERY DISSECTION

• Most common cause of stroke in young adults

• Extracranial ICA most common site

• More common in men than women• More common in Winter• Often goes undiagnosed

PATHOLOGY OF CERVICAL ARTERY DISSECTIONS

• Caused by an intimal tear

• blood dissects along the artery forming an intramural hematoma leading to thrombus

• Vessel occlusion

• Thrombus with subsequent distal emboli

• Pseudoaneurysm formation occurs when the dissection plain lies between the tunica media and adventitia (outer layer)

MECHANISM OF INJURY

• 80% preceded by trauma to the head or neck

• Hyperextension (as in sports, exercise)

• Rotational neck trauma

• Blunt trauma

• Vertebral artery injuries more common

• Direct compression of the neck

• Skull base injury

• Average time from trauma to symptoms is 2-3 days

4

5

6

9/4/2019

3

MECHANICAL CONTRIBUTING EVENTS

• MVC

• Fall

• Chiropractic manipulation

• Exercise–weight lifting, trampoline, impact sports

• Roller coasters

• Can be benign like shaving, yoga, massage, vomiting, coughing

• Can be spontaneous

POSSIBLE CONTRIBUTING MEDICAL COMORBIDITIES

• HTN

• Smoking

• Birth control pills

• FMD

• Ehlers-Danlos syndrome

• Marfan syndrome

• Atherosclerosis

• Migraine history

• infection

• Ipsilateral frontal/temporal headache and neck pain, facial pain

• Partial Horner’s syndrome (Miosis and ptosis without anhidrosis)

• Lower CN palsies (XII, IX, X)

• Diplopia

• Amaurosis fugax

• Ipsilateral Tinnitus (usually “whooshing” sound)

• Audible bruit

• Expanding hematoma/pulsatile neck mass/palpable thrill

• Sudden unilateral decrease in sensation or weakness

• Hypogeusia

• Mean age 47 years

PRESENTATION

CAROTID ARTERY DISSECTION

7

8

9

9/4/2019

4

WHAT YOU MIGHT SEE ON PHYSICAL EXAM

SIGNS TO LOOK FOR• Focal neurologic deficits/stroke

• Ptosis with miosis

• CN palsies

• Cervical bruit

• ”Seat belt sign”

• Signs of cervical spine injury

IX XI XII

VERTEBRAL ARTERY DISSECTION

PRESENTATION• Occipito-cervical pain

• Ipsilateral facial dysesthesia

• Vertigo

• Nausea and vomiting

• Dysarthria (CN IX & X)

• Ipsilateral hypogeusia or ageusia

• Diplopia or oscillopsia

• Ipsilateral hemiplegia

• Contralateral loss of pain and temperature

• Mean age 40.7 years

10

11

12

9/4/2019

5

EXAM

WHAT TO LOOK FOR• Dysmetria

• Limb or truncal ataxia

• Ipsilateral Horner’s

• Contralateral impairment of pain, thermal

• Ipsilateral impairment of fine touch and proprioception

• CN IX & X palsy

CEREBELLAR FINDINGS• Nystagmus

• Tongue deviation to the side of the injury

• Contralateral hemiparesis

• Ophthalmoplegia

• CTA, MRA

• Intimal flaps

• Pseudoaneurysm

• Stenosis or occlusion

• Ultrasound (higher false negatives with injuries closer to the skull base)

• DSA

IMAGING

APPROACH TO DIAGNOSIS

• Initial non contrast CT; if negative, then CTA

• If CTA shows dissection,

• MRI/MRA and

• Referral to neurovascular specialist for DSA

13

14

15

9/4/2019

6

COMPLICATIONS

• Stroke

• Thrombus formation in the false lumen

• Hypoperfusion

• Severe vessel narrowing

• Occlusion of the dissected vessel

• Occlusion of a branch of the vessel by the intimal flap

• Pseudoaneurysm formation

TREATMENT

• Antiplatelets

• Systemic anticoagulation

• Surgical

• Stenting

• Bypass

• Dissections can take 3-6 months to heal

OUTCOMES

• Usually good

• Infarction

• Generally occurs in the first 2 weeks

• Majority have good long term outcome

• Pseudoaneurysms

• Rupture risk about 1%

• Causes SAH

16

17

18

9/4/2019

7

BASILAR ARTERY THROMBOSIS

• Symptoms may be stuttering and wax and wane over weeks to months

• Often mimic other neurologic syndromes like BPV, delirium, coma

CAUSES

• Large-artery atherosclerosis (most common)

• Embolism

• Penetrating small artery disease

• Dissection

• Cervical spine or skull base fracture

• Arteritis

• Aneurysms

PRESENTATION

• Disequilibrium

• Vertigo, nausea, vomiting

• Dysarthria –scanning or staccato speech

• Visual disurbances

• Headache

• Alternating paresthesias

• Tinnitus

• Dysphagia

• Drop attacks

• Ataxia and intention tremor

• Perioral numbness

19

20

21

9/4/2019

8

WHAT TO LOOK FOR ON EXAM

• Test for dysmetria

• Finger to nose, heel to shin

• EOMs—look for nystagmus

• Dysdiadochokinesia (slow alternating movements)

• Gait test—heel to toe, stand on one leg

• Pupil abnormalities

• Oculomotor signs

• Facial weakness, dysphonia, dysarthria, dysphagia

DIAGNOSIS AND TREATMENT

• MRI (assesses for acute stroke)

• CTA/MRA

• Thrombectomy

• tPA

• Antiplatelets

• Warfarin

ARTERIOVENOUS FISTULAS

Abnormal connection between and artery and vein

22

23

24

9/4/2019

9

DURAL ARTERIOVENOUS FISTULA

• Acquired lesions involving an intracranial venous sinus

• Most commonly present at age 40-60

• Fistula forms between the meningeal arteries and veins

• Commonly occurs in the cavernous sinus

CAUSES

• Traumatic head injury

• Infection

• Previous neurologic surgery (craniotomy)

• Tumors

• Progressive stenosis of a venous sinus

• Genetic risk factors

• Predisposition to vein thrombosis

• Benign meningiomas

PRESENTATION

• Highly variable and dependent upon location and anatomy of the lesion

• Pulsatile tinnitus most common

• Bruit (skull/mastoid process)

• Should raise a high suspicion for a vascular lesion

• Headache

• Visual disturbances

• Hydrocephalus may be present as a result of venous hypertension in the superior sagittal sinus which interferes with CSF absorption

• Intracranial venous hypertension can mimic

• Progressive dementia, bithalamic dementia

• Pseudotumor cerebri

• Parkinsonism

• Cervical myelopathy

25

26

27

9/4/2019

10

SPECIFIC PHYSICAL EXAM FINDINGS

• Bruit present

• Cranial neuropathies

• Signs of intracranial hypertension

• papilledema

DIAGNOSIS

• Catheter angiography is the best

• CT scan is not useful

• MRI/MRA is useful in both subjective (only the patient can hear it) and objective (bruit is present) tinnitus

• White matter edema=venous congestions

• Can see hydrocephalus if present

• May give information about the drainage

MANAGEMENT

• Minimally symptomatic dAVF are managed conservatively and expectantly

• Endovascular treatment

• Surgery to interrupt the draining vein

• Stereotactic radiosurgery for those who cannot undergo endovascular or surgical treatment

28

29

30

9/4/2019

11

CAROTID CAVERNOUS FISTULA

Abnormal connection between the carotidartery and/or its branches and a large vein called the cavernous sinus.

The cavernous sinus is located behind the eye and receives blood from brain, orbit, and pituitary gland.

• Communication between cavernous segment of the ICA and cavernous sinus due to a defect in the wall of the ICA (usually a single hole 2-6 mm)

• Usually caused by some type of trauma

• Can be iatrogenic as a result of surgery or neurointerventional procedure

• Endoscopic sinus surgery

• Transphenoidal pituitary surgery

• Balloon rhizotomy

• Pipeline Flow Diversion for cavernous aneurysm

• High flow

TWO TYPES OF CCFDIRECT

• Connection between the cavernous sinus and meningeal branches of the ICA, ECA or both

• Mostly women in their 60s and 70s

• Slight propensity to be on the left

• Risk factors

• HTN, atherosclerotic vascular disease, pregnancy, Ehlers-Danlos syndrome, minor trauma

• Slow flow

INDIRECT

31

32

33

9/4/2019

12

PRESENTATION

• Influenced by the size and type of fistula, location, flow rate, drainage route

• Ocular – can be subtle

• Chemosis, proptosis, eye discomfort

• Cranial nerve palsy (6, 3 and rarely 7)

• Elevated IOP

• Diplopia

• As fistula worsens, periorbital or retrobulbar discomfort, facial pain

• Pulsatile Tinnitus

• Cranial neuropathies

• Facial or ocular motor nerve paresis

• Hemi-facial spasm, TN

PHYSICAL EXAM

• Examine eyes

• Proptosis, chemosis, eyelid edema, ocular pulsations, bruit

• Look for facial pain in trigeminal nerve distribution

• Diplopia

• Optic disc swelling

• Intra-retinal hemorrhage

DIAGNOSIS AND TREATMENT

• Catheter angiogram is the definitive test

• If a fistula is suspected non-invasive imaging:

• CT, CTA

• MRI with contrast concentrated on the cavernous sinus

• MRA

• Specify you suspect a fistula when ordering

• Neuro-ophthalmology referral

34

35

36

9/4/2019

13

TREATMENT

• Treatment is generally endovascular and VERY safe

• Fistula closed (keeping the artery open) with glue or coils

• Accessing the vein through the orbit

• Carotid sacrifice

SPINAL FISTULAS

• Usually starts asymptomatic

• Cord swelling gradually develops

• Problems with leg weakness, back pain, leg pain and bowel and bladder

• Causes generally unknown

• Usually identified by MRI which will show large veins surrounding the cord

• DSA for definitive diagnosis

TREATMENT

• Endovascular embolization

• Surgical

• Outcomes are usually very good

37

38

39

9/4/2019

14

VENOUS SINUS THROMBOSIS

• Clinical symptoms and signs vary

• Affects all age groups

• Large sinuses (eg, superior sagittal sinus) most frequently involved

• Venous system has extensive collaterals and compensates early

• Systemic inflammatory diseases and coagulation disorders are common causes

• Oral BCP

• Continuing process; thrombus progresses over time

PRECIPITATING FACTORS

• Connective tissue diseases

• Granulomatous or inflammatory disorders and malignancies

• 6 weeks post partum

• Oral contraception use

• Prothrombotic conditions

• FacorV Leiden

• Protein C and S deficiency

PRESENTATION

• Symptoms usually appear gradually

• Headache (70-90%)

• Papilledema

• Focal deficits

• More common in women

• Seizure

• Coma

40

41

42

9/4/2019

15

WHAT TO LOOK FOR ONPHYSICAL EXAM

DIAGNOSIS

• MRI with venography (MRV) or CTV

• DSA

• LP to rule out meningitis, SAH, IICH

• Tests to evaluate for the underlying cause:

• Infection

• Head injury

• Malignancies

TREATMENT

• IV heparin

• Local thrombolysis if patient decompensates

• Oral anticoagulation 3-6 months

• Favorable prognosis

43

44

45

9/4/2019

16

CEREBRAL SMALL VESSEL DISEASE

• Diseases affecting the small arteries, arterioles, venules and capillaries of the brain

• Reduced cerebral blood flow, impaired autoregulation, increased BBB permeability

• Leading cause of cognitive decline and functional loss in elderly patients

• Most common forms are cerebral amyloid angiopathy and hypertension related SVD

Small vessels comprise 2 components:

• Leptomeninges vasoganglion (derived from the subarachnoid space covering) and the convex surface of the brain

• Perforating arteries from ACA, MCA and PCA

HYPERTENSIVE SVD

• Age related, most common in elderly

• Exacerbated by HTN, DM

• Vessels become elongated, tortuous and inflexible due to wall damage

• Autoregulation is impaired leading to reduced CBF and chronic hypoperfusion

• Acute occlusion of the arterial lumen leads to acute ischemia and lacunar infarcts

• Severe stenosis and hypoperfusion causes incomplete ischemia seen as white matter hyperintensities on imaging

• Often asymptomatic

• Progressive accumulation of amyloid proteins in small vessels

• Present in almost all elderly with dementia and 65-85% of those without

• Can lead to cognitive dementia, ICH or other transient neurologic events

• ICH usually spontaneous, usually occurring in patients >60 years

• In patients with ICH, 25-40% have recurrence, highest in the first year, and are associated with a high mortality rate

• Common in elderly with Alzheimer disease and Down syndrome

• Severity is age related

CEREBRAL AMYLOID ANGIOPATHY

46

47

48

9/4/2019

17

PRESENTATION

• Cognitive impairment

• >40% of those presenting with ICH have some degree of dementia

• Cognitive changes can precede ICH

• Can present with progressive dementia with rapid decline over days or weeks

• Perceptual speed, language skills and episodic memory impaired

• Intracranial hemorrhage

• Headache

• Pain generally in the area of the hemorrhage

• Vomiting

TRANSIENT FOCAL NEUROLOGIC EVENTS

“Amyloid Spells”

• Transient events similar to TIAs

• Spreading focal weakness, paresthesiaslasting several minutes

• Symptoms spread smoothly over contiguous body parts

• May be a prodrome for hemorrhage

• Avoid antithrombotics!

Distinguished from TIAs by

• smooth spread of symptoms

• lack of large vessel disease

• presence of microhemorrhages on MRI (GRE)

DIAGNOSTIC TESTING

• Head CT—usually find hemorrhages in the frontal and parietal lobes involving the cortex and subcortical white matter

• MRI—microhemorrhages, evidence of multiple large and small petechial cortical and subcortical hemorrhages, siderosis

• DSA—not always abnormal

49

50

51

9/4/2019

18

TREATMENT

• Basically untreatable

• Manage the ICH if present

• Hematoma evacuation

• Hemorrhage prevention

• Avoid AC, BP control

• In inflammatory forms, immunosuppressive therapy

• Referral to a stroke neurologist is highly suggested

GENETIC TYPES OF SMALL VESSEL DISEASECADASIL

(CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITHSUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY)

• Inherited condition that causes stroke and other impairments

• Progressive dementia

• Mood disorders

• Migraine

• Recurrent subcortical cerebral infarcts

PRESENTATION & COURSE

• Tetrad

• Dementia

• Psychiatric disturbances

• Apathy, major depression

• Migraine with aura that begins in young adulthood

• Recurrent strokes – most common

• Later followed by recurrent TIAs then eventually strokes

• Progressive cognitive impairment

• Frontal lobe impairment, memory impairment with intact language

• Late symptoms include gait apraxia, pseudobulbar palsy, urinary incontinence

52

53

54

9/4/2019

19

PHYSICAL FEATURES

• Variable degrees of weakness

• Variable degrees of sensory deficit

• Gait apraxia

• Pseudobulbar palsy

• Parkinsonism/movement disorders

• Psychomotor retardation

• Apathy

• Depressed affect

• Psychosis

REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME

(RCVS)

• Group of disorders characterized by reversible multifocal narrowing of the cerebral arteries

• Associated with abrupt, severe headaches with or without seizures or neurologic deficits

• Constriction of cerebral arteries

• Predominately affects women mean age 42-44 years

• Is reversible with recovery around 3 months

• Can lead to stroke and hemorrhage

PRESENTATION

• Sudden, intense headache +/- nausea, vomiting, photosensitivity

• Frequently have triggers

• Orgasm, physical exertion, acute stressful or emotional situations, valsalva

• Symptoms related to ischemia from the vasoconstriction:

• Seizures

• Visual changes

• Stroke symptoms

55

56

57

9/4/2019

20

CAUSES/RISK FACTORS/ASSOCIATED CONDITIONS

• Drugs—cocaine

• Alcohol, especially binge drinking

• Anti-depressants -- SSRI

• Nasal decongestants and other vasoconstrictive drugs

• Nicotine patches

• Vasoactive tumors (pheochromocytoma, carcinoid)

• Hypercalcemia

• Head trauma

• Pregnancy

TESTING

• MRI

• Vasogenic edema

• Sulcal hyperintensities on FLAIR

• CTA/MRA/DSA

• multifocal segmental cerebral artery vasoconstriction

• Blood/urine toxicology

• Diagnosis is usually made based on clinical presentation (thunderclap headaches) and angiographic studies

TREATMENT

• Depends upon the severity—may resolve without treatment

• Migraine may be treated with aspirin, Depakote

• Calcium channel blocker

• Verapamil

• Magnesium

• STOP any drugs that may be associated!

58

59

60

9/4/2019

21

VASCULITIS

• Inflammation of the blood vessels

• Causes walls of the vessels to weaken, stretch, thicken, scar--> narrow and burst

• More common in people with autoimmune disorders (lupus, RA) or infectious (Hep B, C)

• Causes

• Immune system attacks blood vessels by mistake

• Frequently unknown

• Infection, allergic reaction, lymphoma, leukemia can trigger the immune system

AFFECTS OF VASCULITIS ON THE NERVOUS SYSTEM

• HA

• Aneurysm

• Thrombosis

• Confusion, dementia

• Abnormal sensation/loss of sensation

• Muscle weakness, paralysis

• Pain

• cerebral edema

• Visual problems

• Seizures

• Aphasia

• Cerebral edema

DIAGNOSIS

• Difficult to diagnose

• Difficult to distinguish from non-inflammatory cause of vasoconstriction

• Inflammatory labs

• CSF

• Brain biopsy

• CT, MRI, DSA, US

61

62

63

9/4/2019

22

TREATMENT

• Immune suppression (steroids)

• Supportive care

• Prevention

CENTRAL RETINAL ARTERY OCCLUSION (CRAO)

• Acute stroke of the eye

• Risk factors

• HTN, DM, CAD, Coronary artery disease, TIAs or CVAs, smoking

• Family h/o vascular disease, proatherogenic states

• Increased IOP, optic nerve head drusen, pre-retinal arterial loops

PRESENTATION

• Sudden, painless monocular vision loss

• Unilateral

• History of atherosclerotic disease and/or risk factors

• HTN, atrial fibrillation

• Immediate ophthalmology consult

• Acute: attempt to restore perfusion to the CRA

• Orbital massage

• Decrease IOP

• Acetazolamide, mannitol

• Vasodilators

• Pentoxifylline, nitroglycerine, isosorbide

• Prevent secondary complications to the eye

• Prevent other vascular events in the future

MANAGEMENT

64

65

66

9/4/2019

23

FINAL THOUGHTS

• History is VERY, VERY, VERY important

• A good neurologic exam is critical to picking up subtle (and not so subtle!) findings

• If you suspect a vascular issue, initiate a referral to stroke neurology

67

68