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Diagnosis

HISTORYHISTORY

57 years old Malay lady

underlying Mixed Mitral Valve Disease complicated with Atrial Flutter-Fibrillation not on warfarin.

Admitted on 1st July 2004

Complaint of :

1) Progressive lower limbs weakness x 3/7 prior to admission

2) Progressive inability to swallow x 3/7 prior to admission

3) Changes in voices following onset of inability to swallow

4)Dizziness instability

Further history: On 28th June 2004 i.e; 3/7 prior to admission:1) Patient started to have gradual onset of

lower limbs weakness.She claimed that she felt weak to walk but

still able to walk with assistance. The weakness become more prominence on the day of admission. It is confined to the left lower limb rather than the right lower limb.

Later on she also noted that her left upper limb become weaker.

It is associated with left sided numbness.

2) Patient also noted that she gradually feel difficulty to swallow on the same day of lower limbs weakness.

She claimed that there is no difference in swallowing either fluid and solid food.

The symptom become worse on the day of admission in which immediately after swallowing she experience nasal regurgitation.

There was also episodes of choking.

3) Patient also claimed that she noted that her voices started to change since the onset of inability to swallow.

She claimed that her speech sounds like nasal speech.

4) Complaining of dizziness (vertigo) on the day of onset of illness which persistence till today.

It is associated with instability to walk. She claimed that she is afraid to walk due to feeling of tendency to fall if she walking.

Denied any preceding history of fever with headache or neck pain

No history of loss of consciousness

No history of similar episodes beforeNo bowel or urinary incontinenceNo history of fever or URTI symptom

Denied preceding history of palpitation or chest tightness

CLINICALCLINICAL

Comfortable,Conscious and alert

GCS 15/15

BP: 139 /9 5

PR 102, AF

Febrile : temperature 38.5 C on admission which settled after 3/7 of IV Cefuroxime and Metronidazole

Lungs: Clear

CVS: S1S2 with loud S1 MDM and PSM at MA; Left parasternal heave

Peripheral pulses irregularly irregularJVP not raised

PA: soft and non-tender

Funduscopy: no hemorrhage no papiloedema No neck stiffness

On day 1 of admission (1st July 2004):

T : 38.9 CNystagmus positiveImpaired past pointingNo dysdiadokinesiaRomberg’s sign positive- Fall to left side

Right VII, IX, X, XII CN Palsy

Tone are all normalPower are all 4/5 except on Right upper limbGeneralized hypereflexiaPlantar is equivocal on left side and

withdrawal on right side

On 2nd day of admission (2rd of July 2004)Temperature settling

Right IX, X, XII CN palsyJaw jerk normalNo more past pointingNo dysdiadokinesia

On 4th day of admission (4th July 2004)New neurological findingsLeft hemiparesis:

power are 4/5

reflexes brisk

plantar upgpoingReduced sensation on left upper limb and

lower limb

On day 5th and 6th of admission (5th and 6th July 2004):

GCS 15/15No chyne stokes breathingNoted nasal speechFunduscopy: no papiloedema no hemorrhage

Right eye ptosisPupil 3 mm reactive to light and equal

bilaterally

Fasciculation of tongue noted on right sideTongue deviated to the leftWeakness of the right soft palateGag reflex absentCranial nerve XI intact

No fatigability noted

No nystagmusNo dysdiadokinesiaNo past pointingRomberg sign positive on Right

Findings confined to left upper and lower limbHypertonia on left Power are 4+/5 on left and 5/5 on rightReflexes are brisk on leftPlantar up-going on left

On day 7th of admission (7th July 2004):

GCS 15/15

No chyne stokes breathing

Noted nasal speech

Right eye ptosis

Pupil on the right side is smaller than the left

No fasciculation of tongue noted Tongue slightly deviated to the left ( improved)Weakness of the right soft palateGag reflex absentOther cranial nerve intact

Proprioception intact

Romberg sign positive on RightAtaxia

Findings confined to left upper and lower limb Hypertonia on left Power are 4+/5 on left and 5/5 on right Reflexes are brisk on left upper limb Areflexia both lower limb Plantar equivacle on left

INVESTIGATIONSINVESTIGATIONSHb: 13.5 TWBC: 7.9 PLT: 230,00

BUSE: 4.0 /138 / 3.7; Creat: 105

RBS: 8.0 (FBS: 5.4)

TG: 1.18 Chol: 5.51

12 lead ECG on 1st July 2004; Atrial flutter-fibrillation

CXR on 1st July 2004: cardiomegaly with clear pulmonary fields

Nerve conduction study on 7th July 2004: normal

ECHO on 8th July 2004:

Moderate MS, Trivial MR, dilated LA (4.13 cm)

no intracardiac clots, no vegetation

CT scan of brain on 1st July 20004 (day 3 of illness) reported as normal.

MRI of brain on 7th July 2004 (day 10 of illness) verbally reported as

1) Evidence of shower of emboli to both hemisphere predominantly on the right side;

2) Right parietal,Right centrum semi ovale

3) Small area in the right cererbellum

4) Right brainstem

ASSESSMENTASSESSMENT

1)Brainstem Strokemost probably posterior circulation

( low flow state) Diff: Variant of Guillen Barre’s Syndrome

(polyneuropathy cranialis)

2)Atrial flutter fibrillation – Rate controlledcausing thrombolic phenomenon

3)Mixed mitral heart disease

4)Aspiration Pneumonia

MANAGEMENTMANAGEMENT

IVI heparin started on 2nd July 2004 adjusted according to APTT ratio. Heparin was off on 6th July 2004.

Oral Warfarin started on 2nd July 2004;

latest INR is 2.33 on 7th July ( on Warfarin 3 mg od).

T Aspirin 150mg OD (started on 7th July 2004)

T Provastatin 20 mg oNT Stegeron 25 mg tdsOral Digoxin 0.25 mg od

T Ranitidine 150 mg bdIV Cefuroxime 750 mg tds (started on 1st

July 2004)IV Metronidazole 500 mg tds (started on 1st

July 2004)

differential DIAGNOSISdifferential DIAGNOSIS

1) Brainstem stroke

2) Polyneuritis Cranialis

3) Variant of GBS – Miller Fisher Syndrome

4) Vilarret’s Syndrome

5) Septic Emboli

DISCUSSIONSDISCUSSIONS

Bulbar palsy results from bilateral impairment of function of the IXth, Xth and XIIth cranial nerves.

This gives rise to dysarthria, dysphagia (often with choking episodes and nasal regurgitation of fluids), dysphonia and poor cough, and susceptibility to aspiration pneumonia.

The lowermost part of VII may, infrequently, be involved.

The disturbance is of the motor nuclei rather than of the corticobulbar tracts. It is distinguished from pseudobulbar palsy by the presence of lower motor neurone signs. Autonomic features are uncommon.

Pseudobulbar Palsy VS Bulbar Palsy

1) degeneration or disturbance to :P: X, XI, XII, corticobulbar pathways

sometimes VIIB: rather than the to V,VII,X,XI,XII

corticobulbar tracts

2) lower motor neurone signs P: absent B: present

3) Gag reflexP: (+/n) B: (-)

4) spastic tongue wasted tongue, fasciculations

jaw jerk (+) jaw jerk (n) spastic dysarthria nasal speech labile emotions normal emotions bilateral UMN signs in limbs+ = increased; - = reduced; n = normal

Posterior cerebral artery territory infarcts

Infarcts in the territory of the posterior cerebral arteries (PCAs) are common.

Although associated clinical symptoms and signs are known, the mechanisms of stroke and the anatomical distribution of PCA territory lesions caused by the various stroke mechanisms are less well defined.

Forty-eight patients (61%) had infarcts limited to the PCA territory (pure PCA), while 31 (39%) also had infarcts in other territories (PCA+).

Infarcts were in the cortical territory of the PCA in 47 patients (59%) and were cortical and deep in 32 (41%). Infarcts that were cortical and deep were more common in PCA+ lesions

Stroke mechanisms were embolism of cardiac origin (32 [41%]), proximal arterial disease (25[32%]), cryptogenic embolism (8[10%]), intrinsic PCA disease (7[9%]), vasoconstriction (4[5%]), and coagulopathy (3[4%]).

Patients with cardiogenic embolism and intrinsic PCA disease often had pure PCA territory infarcts, while patients with proximal arterial disease more often had PCA+ infarcts. Visual abnormalities were present in 66 patients (84%). Motor weakness, cognitive and behavioral abnormalities, and ataxia were found in 20 patients (25%); only 12 (15%) had sensory signs.

CONCLUSIONS: The great majority of pure PCA and PCA+ territory infarcts are caused by cardiac or intra-arterial embolism. Intrinsic PCA disease, vasoconstriction, and coagulopathy are less common causes of infarction.

Villaret's syndrome

Synonyms:Parotid space syndrome, posterior retroparotid space syndrome.

A syndrome of ipsilateral paralysis of the ninth, tenth, eleventh, twelfth, and sometimes the seventh cranial nerves and the cervical sympathetic fibers.

It is caused by a lesion in the posterior retroparotid space.

The clinical manifestations include Horner's syndrome and paralysis of the soft palate, pharynx, and vocal cords.

In some cases there also may be paralysis of the superior constrictors of the pharynx, numbness of the soft palate, fauces, and larynx, loss of taste of the posterior one third of the tongue, and paralysis of the sternocleidomastoid and trapezius muscles.

Collet-Sicard syndrome Collective term comprising infectious disorders associated with encephalitis epidemica.

Villaret's syndrome A syndrome of ipsilateral paralysis of the ninth, tenth, eleventh, twelfth, and sometimes the seventh cranial nerves and the cervical sympathetic fibers.

Miller-Fisher SyndromeMiller-Fisher SyndromeThe Miller-Fisher syndrome triad

– Ophthalmoplegia– Ataxia– Areflexia

CSF protein is usually is elevated, but the NCS findings are more suggestive of axon loss than SD

It is generally a benign disorder and does not require specific immune therapy

A pure sensory form has been reported– The diagnostic criteria for GBS proposed in

1978 exclude this variant

Vasculitic NeuropathiesVasculitic Neuropathies Vasculitic neuropathies occasionally are mistaken

for GBS Distinctions:

– Systemic signs, including fever, are common– Sensory and motor involvement in the limbs usually is

both distal in location and asymmetrical– Cranial nerve involvement, respiratory complications,

and sphincter dysfunction are uncommon– CSF typically is normal (except with systemic lupus

erythematosus)– NCS reveal changes suggestive of axon loss, rather

than SD