Moyamoya disease is a chronic cerebrovascular disease characterized by stenosis or occlusion at the terminal portion of the internal carotid artery,accompanied by the formation of an abnormal vascular network.If these cerebral angiography findings are only seen in one hemisphere of the brain,it is unilateral moyamoya disease(U-MMD).Numerous studies have shown that U-MMD is not uncommon.The latest version of diagnostic criteria has clearly diagnosed U-MMD as Moyamoya disease,rather than being a"possible"moyamoya disease.The pathogenesis of this disease involves genetic,immune,and environmental factors,but the exact cause is currently unclear.Compared with the bilateral moyamoya disease,the U-MMD has different clinical features and imaging manifestations,and has better surgical outcome,and many risk factors promote its progression to the contralateral side.This article reviews the epidemiology,pathogenesis,clinical characteristics,treatment,and surgical outcome of U-MMD,and looks forward to the future research directions.
Moyamoya diseaseNeuroimagingGenetic predisposition to diseaseDisease progressionCerebral revascularizationTreatment outcome
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