Iranian Journal of Child Neurology

Abstract

Objective

Moyamoya disease (MMD) is a chronic, occlusive, cerebrovascular disorder of unknown  pathogenesis, characterized by progressive stenosis of the bilateral supraclinoid internal carotid arteries, with concomitant formation of tortuous arterial collateral vessels at the base of the brain, which reconstitute distal branches of the cerebral circulation. In Japanese, "Moyamoya" means "hazy puff of smoke" and refers to the angiographic appearance of the abnormal network of vessels that develop at the base of the brain and basal ganglia to supply a collateral route of blood flow. We report here the case of Moyamoya disease in a 5 year-old girl with normal mentality with a one year history of epilepsy, with Todd's paralysis. This condition is rare and most patients are diagnosed in childhood. With this report we aim to underscore the possibility that a usual neurological sign could be associated with unusual neurological disorders.