Moyamoya disease: a silent smoke

Abstract

Moyamoya disease (MMD) is an isolated chronic, usually bilateral, vasculopathy of undetermined etiology characterized by progressive narrowing of the terminal intracranial portion of the internal carotid artery and circle of Willis. It is a connective tissue disorder of cerebrovascular vessels of unknown aetiology. A fragile network of abundant collateral vessels as a reaction to chronic brain ischemia develops predominantly at the base of the brain known as moyamoya vessels. This activity describes the pathophysiology, evaluation, and management of MMD and highlights the role of the interprofessional team in the management of affected patients. Here we present a case of 3-year-old female child who had frequent transient episodes of twitching of left side of her face with deviation of angle of mouth towards right side since 15 days without neurological deficit and altered sensorium. There also was a history of transient episode of left sided paresis at the age of 18 months of life.  Detailed evaluation and magnetic resonance imaging (MRI) confirmed the diagnosis of MMD. In this particular case the age group of presentation for MMD is unusual. Discussion of this case will help to spread awareness about early suspicion and appropriate intervention at right time to improve the developmental and cognitive status of such cases.