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The Periodical Second Issue, November 2002 |
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| 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.