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Open Access Case Report

Fluctuating Hemiparesis Secondary to Moyamoya Phenomenon in a Child with Down Syndrome: a case report

Richard A Rison

Author Affiliations

Clinical Assistant Professor of Neurology, University of Southern California, Keck School of Medicine, Los Angeles County Medical Center, Neurology Consultants Medical Group, 12291 E. Washington Blvd. Suite #303, Whittier, California, 90606, USA

Cases Journal 2008, 1:240  doi:10.1186/1757-1626-1-240

Published: 15 October 2008

Abstract

Moyamoya phenomenon is a term used to describe extensive collateralization of the circle of Willis arteries associated with severe unilateral or bilateral internal carotid artery stenosis or occlusion in the presence of certain conditions. Down syndrome is among these conditions. A case is reported of a young girl with Down syndrome who presented with fluctuating right-sided weakness and facial droop found to have cerebral ischemia. Subsequent investigations disclosed characteristic "puff of smoke" patterns on angiographic studies consistent with moyamoya phenomenon. The patient was initially treated with aspirin and eventually underwent an encephalomyosynangiosis. This young patient with Down syndrome and moyamoya phenomenon serves as a reminder of the association between these two conditions.