The Periodical Second Issue, November 2002

 

 

Editors Note SAGAS Objectives SAGAS News Stroke Risk Factors: An Overview
A Need For A World Stroke Federation International Article Review The Periodical Quiz The Periodical Case

The Periodical Case

A Case of Moyamoya

 22 year old Saudi single private male  

First ever:

Sudden and mild, left upper limb weakness with facial tilt  

Negative history for:

Headache, LOC, seizures, speech difficulties, diplopia, dysphagia, sensory changes, sphincters loss, abnormal movements and head or neck trauma  

No risk factors and no drug abuse 

Negative family history 

Negative systemic inquiry 

 

Examination:

Obese young man, who is slightly dull with mild left hemiparesis. No other abnormalities

Neuro-psychological testing:

Below average performance at school and later at work. Impaired attention and concentration with mild depressive symptoms 

IQ:

Verbal IQ 87

Performance IQ 90

Full IQ 88

Memory:

Verbal, mild deterioration

Visual, normal

Global memory function 85%

 

Work up

CT scan of the brain shows oval hypodensity in the right frontal watershed area, which is not enhancing with contrast

 

CXR, ECG, ECHO and TEE are normal.

Hemogram, biochemistry, inflammatory reactants, lipid profile, pyruvate and lactate and all hypercoaguble parameters are normal

Sickle cell and autoimmune screen are negative

CSF examination is normal

Evoked potentials are normal

 

TCD

Bilateral ICA distal severe stenosis and severe stenosis of both ACA and MCA bilaterally with plenty of collaterals and normal posterior circulation (suggesting moyamoya disease)

 

 

 

 

 

EEG

Shows rebuild of frontal high amplitude delta wave, 2 minutes following stopping hyperventilation.

 

Angiography

Severe stenosis of distal internal carotid artries bilaterally, with severe stenosis of both middle cerebral and anterior cerebral arteries bilaterally with compensated dilated lentriculo-striatal arteries (moyamoya phenomena, stage III-IV). The basialr artery is normal.

  AP view

  Lateral view

 

SPECT Scan

shows low perfusion in frontal areas, more on the left.

 

Treatment

Medical

ASA and Verapamil

Surgical

Bilateral encephalo-myo-synangiosis (EMS)

Follow up

The 1 year follow up shows improvement of neurological deficit with no stroke recurrence. His mental and psychological state improved:

IQ 92

Memory is unchanged

Mood improvement

Case discussion

 The recent literature support the intervention with surgical procedures, to reduce the risk of stroke (ischemic or hemorrhegic).

Send an e mail about what do you think about this case and whether the management is appropriate.