Introduction: Moyamoya disease(MMD) is an idiopathic cerebrovascular disease characterized by progressive stenosis or occlusion of the bilateral internal carotid arteries and formation of abnormal collateral vessels at the base of brain. A large number of cases of MMD in China were sporadic compared with approximately 15% familial incidence in Japan.The relationship between geographic distribution and incidence of MMD was unclear.We performed this study to discover the region with a high incidence of moyamoya disease and to depict the unique epidemiological and clinical features that may explain the etiology.
Methods: Three villages were randomly recruited according to regional distribution of moyamoy disease in Zhuji City,Zhejiang Province between Jan 2013 and Oct 2013. Detailed questionnaires were designed including general information, lifestyle, environment and medical history.1.5T magnetic resonance imaging(MRA) was applied in all of the participants for detecting MMD.Epidemiological and clinical feature of MMD patient were obtained and analyzed.
Results: 537 participants were included from Shenhe,Dayuan and Baima Villages.14 MMD patients were identified by MRA,who were discovered in the same village,namely Shenhe Village.Detection rate of MMD was 2.6%,which was extremely high. No familial history of the disease was observed.Their ages ranged from 31 to 62 years(mean 52) and 8(57%) were female.9(64%) were hemorrhagic type,4(29%) were asymptomatic type and 1(7%) was ischemic type.Lifestyle and environment had no statistical difference with MMD(P>0.05).
Conclusions: A village with a high detection rate of MMD was first discovered in China and hemorrhagic type predominated over other types,which implied that genetic factors may play an important role in the etiology of MMD.
Patient Care: Discovery of the region with high-prevalance of moyamoya disease would help elucidate the etiology.
Learning Objectives: By the conclusion of this session,participants should be able to know that a village with a high detection rate of MMD was first discovered in China and hemorrhagic type predominated over other types,which implied that genetic factors may play an important role in the etiology of MMD.