Moyamoya Disease: From Genetics To Clinical Management

Moyamoya angiopathy is characterized by a Chronic progressive angiopathy with the Circle of Willis involvement of anterior circulation and the development of a network of abnormal collateral vessels.This chronic cerebral angiopathy occurs in both children and adults.moyamoya disease is defined as being “idiopathic” according to the Guidelines of the Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle of Willis.This entity is probably multifactorial and polygenic in most patients.moyamoya syndrome is a moyamoya angiopathy associated with basic disease.It has many clinical manifestations and genetic patterns.Clinical manifestation of moyamoya disease can be divided into two types: cerebral hemorrhage and cerebral ischemia.It mainly leads to brain ischemic events in children,and to ischemic and hemorrhagic events in adults.This is a rare condition,And there are regional differences in the incidence of the disease.It has been reported that there are significant differences between Asian countries and other countries,such as the West.Diagnostic and evaluation techniques rely on magnetic resonance imaging(MRI),magnetic resonance angiography(MRA),conventional angiography,and cerebral hemodynamics measurements.Revascularization surgery is seen to the main clinical treatment.This article reviews the progress of gene research on moyamoya disease,clinical onset and characteristics,clinical evaluation,diagnosis and treatment of moyamoya disease.