ABSTRACT
Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease of unknown etiology reported mainly in the Japanese. Most cases occur in children. The disease is rare in non-Oriental adults manifesting itself mostly as intracerebral hemorrhages. We describe MMD in 2 non-Oriental young adults and one adolescent that developed cerebral infarctions. The adults were medicated with aspirin and no medication was given to the adolescent. All patients did not deteriorate in a follow-up period from 1 to 4 years. Although rare, MMD is na important cause of stroke in young individuals and may well be underreported: only 18 patients have been reported till 1997 in Brazil. Neurologists should include MMD in differential diagnosis of ischemic and hemorrhagic strokes in young adults.
Subject(s)
Humans , Male , Adolescent , Adult , Moyamoya Disease/diagnosis , Aspirin/therapeutic use , Brazil , Cerebral Infarction , Follow-Up Studies , Moyamoya Disease , Moyamoya Disease/drug therapy , Platelet Aggregation Inhibitors/therapeutic useABSTRACT
Relato de caso de neurofibromatose tipo I associada a doença oclusiva extensa do sistema carotídeo em sua porçao intracraniana e aneurisma fusiforme de circulaçao posterior. O paciente, de 28 anos de idade e com diagnóstico de doença de von Recklinghausen, passou a apresentar episódios de síncope, crises parciais complexas e declínio cognitivo. Após quadro agudo de cefaléia e sinais de irritaçao meníngea, com líquor hemorrágico, o paciente foi investigado com, TC de crânio, RNM e angiografia cerebral, sendo detectadas alteraçoes tipo moyamoya e dilataçao aneurismática de artéria cerebral posterior. Apresentamos os achados clínicos e radiológicos deste caso com poucos relatos similares na literatura médica, discutimos opçoes terapêuticas e reiteramos a inclusao de diagnósticos diferenciais raros em indivíduos que apresentem icto com menos de quarenta anos de idade.