Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan

Abstract

BackgroundMoyamoya is a rare cerebrovascular disease of unknown etiology. The data on moyamoya disease from Pakistan is sparse. We report a case series of 13 patients who presented with moyamoya disease to a tertiary care hospital in Pakistan with a national referral base.MethodsWe conducted a retrospective review of thirteen patients who presented to The Aga Khan University and diagnosed with "Moyamoya Disease" during the period 1988 – 2006. These patients were identified from existing hospital database via ICD-9 codes. A predesigned questionnaire containing information about clinical presentation, management and neuroimaging was administered to all identified patients.ResultsThere were seven males and six females. Mean age at presentation was 16.5 years and a female predominance was found in the pediatric age group (n = 10, 71.4%). Stroke (n = 11, 84.2%) was the most common presentation with motor deficit being the universal cortical symptom. Fever was a common symptom in the lower age groups (n = 4, 51.7%). Cerebral Angiography and Magnetic Resonance Angiography showed bilateral involvement of the vessels in eleven patients while unilateral in two. Subarachnoid and interventricular haemorrhage appeared in 2(15.4%) adults. Twelve (92.3%) patients were discharged as independent with minor deficits regardless of therapeutic modality. Only three (23.0%) patients underwent surgery whereas the remaining were managed conservatively.ConclusionPhysicians when dealing with childhood strokes and characteristic deficits in adult population should consider Moyamoya disease.

Clinicoradiological Profile of Childhood Moyamoya Disease: Indian Study of 30 Children with Literature Review

“…All patients in the studies conducted by Singhi et al 21 The only patient with hemorrhagic stroke presented with basal ganglia bleed with intraventricular hemorrhage. Duan et al 26 had 2.3% incidence of hemorrhagic stroke in the pediatric population, whereas Shoukat et al, 19 Srivastava et al 23 and Singhi et al 21 had none. Historically, bleeding has been attributed to rupture of fragile collateral vessels associated with moyamoya as progressive stenosis of the ICA occurs.…”

Electroencephalographic features of moyamoya in adults

“…One small mixed series (Shoukat et al, 2009) of seven patients under age 12 years and six over age 12 reported EEGs in six patients: four with diffuse slowing and two with epileptiform activity, but the age of the patients receiving EEGs was not specified. Similar observations were made in an Italian series of 27 pediatric and 7 adult cases with moyamoya.…”

Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome

“…There has not been much studied regarding moyamoya in our population as there are only two case series with a total of 17 patients, of which 10 were of less than 12 years of age [3, 4]. Few studies reported higher incidence of stroke in younger age group in our part of the world as compared to western population, which probably indicate underdiagnosis of moyamoya [5].…”