• Peicong Ge, Qian Zhang, Xun Ye, Xingju Liu, Xiaofeng Deng, Hao Li, Rong Wang, Yan Zhang, Dong Zhang, Yong Cao, Wang Shuo S Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing; China National Clinical Research Center for Neurological Diseases, , and Jizong Zhao.
• Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing; China National Clinical Research Center for Neurological Diseases, Beijing; Center of Stroke, Beijing Institute for Brain Disorders, Beijing; Beijing Translational Engineering Center for 3D Printer in Clinical Neuroscience, Beijing; Beijing Key Laboratory of Translational Medicine for Cerebrovascular Disease, Beijing, China.
• World Neurosurg. 2017 Oct 1; 106: 224-230.

ObjectiveTo elucidate the clinical features of patients with hemorrhagic moyamoya disease (MMD) in China.MethodsWe retrospectively reviewed 471 patients with hemorrhagic MMD at Beijing Tiantan Hospital. Clinical features and radiologic findings were analyzed.ResultsThe mean age at diagnosis was 35.3 ± 11.5 years, with 1 peak distribution in patients from 35 to 39 years of age. The ratio of women to men was 1.2:1. Familial occurrence was 3.8%. The primary symptoms at initial presentation were intraventricular hemorrhage (42.0%), intracerebral hemorrhage (23.6%), intracerebral hemorrhage with intraventricular hemorrhage (18.3%), and subarachnoid hemorrhage (16.1%). Before the diagnosis, 68 patients experienced a second episode of bleeding. Rebleeding tends to be common within 6 years after the first bleeding (83.8%). The second bleeding episode was characterized by a change in which hemisphere bleeding occurred in 7 patients (10.3%) and by the type of bleeding in 23 patients (33.8%). Most patients presented with Suzuki stage 3 or 4 MMD (61.7%). Posterior cerebral artery involvement was observed in 28 (18.4%) patients. Forty-three intracranial aneurysms were identified in 39 patients (8.3%).ConclusionsA 1-peak pattern in age distribution and mild female dominance in sex distribution were observed in patients with hemorrhagic MMD. Rebleeding tends to be common within 6 years after the first bleeding; however, some cases of rebleeding occur after a long period. Furthermore, the second bleeding episode was characterized frequently by a change in hemisphere and the type of bleeding. Early surgical treatment in both hemispheres is recommended.Copyright © 2017 Elsevier Inc. All rights reserved.

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