Moyamoya Disease: Case Report

Moyamoya disease is a condition characterized by progressive narrowing of Intracranial arteries around Circle of Willis and development of collaterals. This case report describes a 12-year-old Ukrainian Girl, migrated to Ireland couple of months ago, presented with right sided facial palsy and right hemiparesis of sudden onset. Her MRI brain revealed acute infarct in MCA territory with loss of flow in both proximal and middle cerebral arteries. CT Angiogram showed abnormal appearance of ICA and circle of Willis. progressive narrowing of right MCA and high-grade narrowing of left MCA suggestive of Moyamoya disease. Conservative management plan was decided Prednisolone, aspirin in tapering dose was started, she showed complete recovery in couple of weeks. There was no underlying association with any haematological, immunological or infectious cause was identified and there was no family history of such event. Moyamoya disease has mortality rate of 4.5% is children and majority of children develop gradual cognitive decline over time.