ABSTRACT
Moyamoya disease is rare, progressive, occlusive cerebrovascular disease with characteristic angiographic bilateral stenosis or occlusion of the distal internal cerebral arteries with compensatory development of a fine vascular network at the base of the brain. The etiology and pathogenesis are unknown. The disease shows a familial incidence, and mutifactorial inheritance has been suspected. The diagnosis of Moyamoya disease is made on the basis of angiographic fingings: In its early stage there is stenosis of the supraclinoid portion of the internal carotid artery, frequently bilaterally. Later there is enlargement of extracranial transdural collateral circulation. Clinically, this disease is found with a higher incidence in young female, but there are few cases of Moyamoya disease in pregnancy. The disease usually presents in children with paroxysmal hemiplegia, hemiparesis, involuntary movement, headache, and seizures. In adults of reproductive age the disease usually occurs as alterations of consciousness caused by cerebral ischemia or intracranial hemorrhage. We report a case of Moyamoya disease that presented as cerebral infarction during pregnancy complicating severe preeclampsia.