[Moya-moya syndrome. Progression of the angiographic picture and therapeutic prospectives].

Moya moya is an obstructive cerebrovascular disease characterised by peculiar cerebral angiographic features consisting of intracranial stenosis or occlusion of the internal carotid artery or its terminal branches associated with telangiectatic vessels at the base of the brain. Opinion is still divided between a congenital versus an acquired aetiology. Recurrent episodes of sudden hemiplegia, headache and convulsive manifestations occur more frequently in paediatric patients, while subarachnoid bleeding is usually the presenting finding in adults. After a progressive course for many years, the disease frequently stabilizes sometimes with residual disability. Surgical approach to improve cerebral blood flow has been developed but there is a high incidence of complications in pediatric patients. To minimize the defects during ischemic phase, a pharmacological therapy might be useful. We report a new case of childhood moya moya disease which developed a typical angiographic progressive pattern during three years between the first and the latest bilateral study. Clinical course of patient suggest that antiaggregating drugs and calcium antagonists might be useful in this disease although controlled studies are of course needed.