Sandhya Manorenj

By Sandhya Manorenj and Reshma Sultana Shaik

Moyamoya disease is a chronic progressive, non-atherosclerotic, occlusive intracranial vasculopathy involving major cerebral arteries around the circle of Willis. MMD occurs frequently in East Asian populations but the disease can affect the American and European ethnicities as well. Knowledge of clinical aspects of Moyamoya disease (MMD) is important in view of distinctive clinical presentation observed in children and adults. MMD has bimodal age of distribution, with peaks in the first and last decades of life. Childhood MMD is characterised by Ischemic manifestation (Transient ischemic attack, Cerebral Infarction), whereas adult MMD presents with hemorrhagic manifestations (Intracerebral haemorrhage, Intraventricular bleed). Refractory headache, seizure and ophthalmological abnormalities are other clinical presentations of MMD. A high index of clinical suspicion and an eye to recognise the common as well as unusual manifestations of the disease and inciting events may prevent delay in the diagnosis. A thorough knowledge about the varied clinical presentation would aid clinician for early diagnosis and management of this rare entity. The present article provides extensive review on the clinical aspects of MMD amongst adults and paediatric population, on the basis of previous articles and research studies.

Part of the book: Moyamoya Disease