Epidemiological Survey Of Moyamoya Disease And Clinical Treatment

Part 1: Epidemiological and clinical features of Moyamoya disease in Nanjing, ChinaObjective The epidemiology of Moyamoya disease in mainland China has not been documented. Therefore, the present study was designed to examine the epidemiological and clinical features of Moyamoya disease in Nanjing, a provincial capital in China. Methods Patient records from multiple hospitals in Nanjing from January 2000 to December 2007 were collected. The clinical features of Moyamoya disease were retrospectively analyzed. Results A total of 202 patients were identified. There were 94 males and 108 females, with ages ranging from 2 to 78 years. There was a dual age peak, one in the group of patients 5–9 years of age and another in the group of patients 35–39 years of age. The initial symptoms included cerebral ischemia (81 patients, 40%), cerebral hemorrhage (113 patients, 55.9%) and asymptomatic disease (8 patients, 3.9%). An increasing incidence rate of Moyamoya disease was observed during the period of 2000–2007, with an average detection rate of 0.43 cases/100,000 persons/year (prevalence 3.92/100,000 persons). The incidence of ischemia associated with the disease was 0.16 cases/100,000 people-years and the incidence of hemorrhage was 0.22 cases/100,000 people-years.Conclusion This first study on the epidemiological and clinical features of Moyamoya disease in mainland China indicated an increasing incidence of Moyamoya disease with bimodal incidence distribution appearing more frequently in adults. Part 2: Observations on the clinical effectiveness of Indirect revascularization (EDAMS) on Moyamoya diseaseObjective To explore the therapeutic effect of encephaloduromyosynangiosis(EDAMS)in treatment of moyamoya disease. Methods 17 patients with moyamoya disease were included from October 2003 to December 2009. EDAMS were performed on the all patients. The clinical manifestations, MRI, MRA were followed and compared between pre- and post-operation to evaluate the clinical effective of EDAMS. Results Of 17 patients with MMD, there were 8 males and 9 females, with an average age of 35.42 years ranging from 2 to 78 years old. The initial symptoms included cerebral ischemia (9 patients), cerebral hemorrhage (8 patients). Therapeutic effect was observed in ischemic patients (77.78% ), hemorrhagic patients(62.5% ), total patients(70.59% ), separately. A follow-up study of average 32.6 months ranging from 6 to 54 months was available. At the end of the follow-up, none of the patients appeared a second hemorrhage. The examination of MRA after surgery showed supplementally extracranial blood supply and the different levels of diminishments of abnormal vascular network in skull base.Conclusions EDAMS is a safe and effective surgery for moyamoya disease. It improves the cerebral blood flow infusing condition and promote the diminishments of moyamoya vessels by establishing the good lateral branch circulation. Long-termed observation on clinical effective should be implemented due to the small sample size and short follow-up duration.