RESUMO
BACKGROUND: Moyamoya disease is a rare idiopathic non-atherosclerotic, non-inflammatory and non-amyloid cerebrovascular disease characterized by stenosis and progressive occlusion of the internal carotid artery and its branches. Upon panangiography, this abnormality is typically visualized as a cigarette smoke-like pattern due to formation of new collateral vessels. The highest incidence occurs in Asia, principally affecting women. This case report describes the characteristics of these patients and the way to diagnose and treat them. CLINICAL CASE: A 43-year-old man with a history of chronic headache, subarachnoid hemorrhage and tonic clonic seizures; because of his age he was programmed to the young patient study protocol and so he underwent some studies like angiography, angiographic resonance and transcranial Doppler, these exams revealed a bilateral stenosis of the internal carotid arteries and its branches. DISCUSSION: The prognosis of these patients is unfavorable when the disease begins in childhood. Since conservative treatment may not be long-term useful, surgery should be considered in patients with Moyamoya disease. The use of transcranial Doppler is recommended in patients with these clinical features as an initial diagnostic exam.