[Hemorrhagic type of moyamoya disease]

Clinical picture of Moya Moya Disease was analyzed in 18 cases. In 16 cases whose CT scan were available at the time of the first bleeding, hematoma at the basal ganglia was noted in 43.8%, primary ventricular hemorrhage in 37.5%, thalamic hemorrhage with ventricular rupture in 12.5% and subcortical hemorrhage in 6.3%. The frequencies shown above were well correlated to previous reports. In MRI performed 1 year or more after primary ventricular hemorrhage, the primary bleeding site was demonstrated at the lateral wall of the lateral ventricle, in proton weighted and T2 weighted images. MRI can detect the site of old bleeding points and its chronological change if the study is repeated. In a follow-up period of 5.4 years, 27.8% of the cases had rebleeding one or more times. As a result, good outcome was noted in 72.2% after the 1st bleeding, and in 55.6% after re-bleedings. Death occurred in 5.6% of patients after the 1st bleeding and in 22.2% after further rebleeding. Rebleeding worsened the outcome. Therefore, prevention of rebleeding is important. From a therapeutic viewpoint, although a direct relation between rebleeding and untreated hypertension could not be established, blood pressure control is critical at both the acute and the chronic stages. Reconstructive vascular surgery is a recommendable method for properly selected patients.