抄録
Four patients with unilateral atypical moyamoya disease, ages 19, 42, 43, and 47 years, presented with intracerebral and/or intraventricular hemorrhage and underwent surgery. Preoperative carotid angiograms of the affected side showed severe stenosis at the terminal portion of the internal carotid artery with development of collaterals via transdural, cortical, and transparenchymal pathways. The contralateral internal carotid arteries were completely normal. Two patients were treated by encephalo-myo-synangiosis (EMS), the third by encephalo-myo-arterio-synangiosis, and the fourth by EMS and superficial temporal-middle cerebral artery anastomosis. Postoperative angiograms revealed good neovascularization through numerous branches of the external carotid artery. The process of neovascularization in these four cases was similar to that observed in typical moyamoya disease. The authors believe that the four cases presented do not represent arteriosclerotic cerebrovascular occlusive disease and should be included in the category of moyamoya disease.
