Moyamoya disease: an elusive diagnosis.
OBJECTIVE
Moyamoya disease is an idiopathic vasculopathy of circle of Willis. Stroke is a common presentation. We describe clinical and radiological features of moyamoya disease in four patients.


METHODS
Tertiary care center in a metropolitan city.


METHODS
Patients with moyamoya disease were identified by ICD-9 coding system of the hospital medical records. Demographic characteristics, clinical features/presentation, laboratory investigations and radiological investigations were recorded and analyzed.


RESULTS
Four patients (three children and one adult) presented with hemiparesis. One patient expired during hospital stay. Two patients underwent surgery (synangiosis). EEGs of three patients showed focal abnormalities. Neuroimaging showed ischemic infarctions in all patients. Findings on four vessel digital substraction angiography and/or magnetic resonance angiography were compatible with moyamoya disease.


CONCLUSIONS
Moyamoya disease should be considered in all young patients, especially children, presenting with stroke.
Asumal
K B
KB
Section of Neurology, Department of Medicine, The Aga Khan University Hospital Karachi.
Akhtar
N
N
Syed
N A
NA
Shafqat
S
S
Baig
S M
SM
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography / Q000379:methods; D008297:Male; D009072:Moyamoya Disease / Q000150:complications / Q000175:diagnosis*; D010154:Pakistan; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D020521:Stroke / Q000175:diagnosis* / Q000209:etiology; D014057:Tomography, X-Ray Computed