关键词: 烟雾病; 脑梗死; 脑出血

Abstract: Objective To explore the clinical features in adult patients with moyamoya disease. Methods The age of onset, gender and clinical features of 244 Chinese adult patients with moyamoya disease were analyzed and compared with the data of the patients with moyamoya disease in Korea, Japan and North America. Results In the 244 patients, the age was from 18 to 72 years old. The mean age was 36 years. The male and female ratio was 1:1.03, which 120 were females and 124 were males. The initial symptom was cerebral ischemia in 187 patients and cerebral hemorrhage in 57 patients. All the patients came from 28 different provinces. Patients from Henan and Hebei province were 34 and 30 cases, which the proportion was the most. All the patients had digital subtraction angiography (DSA)/ magnetic resonance angiography(MRA) examination, and according to Suzuki stage, the I to VI stage was in 24, 96, 112, 152, 80 and 24 hemispheres respectively. In our cases, 244 patients underwent encephaloduro- arterio-synangiosis (EDAS) operation. After the operation, 57 cases that the initial symptom was hemorrhage had no rebleeding, and 161 cases in 187 patients that the initial symptom was ischemia had relief obviously, 26 cases no marked change. Conclusion Adult patients in China with moyamoya disease present with different clinical features compared with other countries. These observations may indicate the various mechanisms involved in moyamoya disease in China. EDAS is an effective method for the adult patients with moyamoya disease.

Key words: Moyamoya disease; Cerebral infarction; Intracranial hemorrhage