Moya moya syndrome is a specific chronic cerebrovascular occlusive disease first reported by Japanese surgeons in 1957. The disease moya moya, which is a Japanese mimetic word, gets its characteristic name due to the appearance of puff of smoke on relevant angiographs resultant from the tangle of tiny vessels in response to stenosis. This makes the blood to leak out of the arteries, causing pressure to the brain. It may cause ischemic attacks or cerebal infarction, which is more frequent in children than in adults. The highest peak is in childhood at less than 10 years of age. The disease causes constrictions primarily in the internal carotid artery, and often extends to the middle and anterior cerebral arteries, branches of the internal carotid artery inside the skull. When the internal carotid artery becomes completely blocked, the fine collateral circulation that it supplies is obliterated. The clinical features are strokes, recurrent transient ischemic attacks (TIAs), sensorimotor paralysis (numbness and paralysis of the extremities), convulsions and/or migraine -like headaches. Moreover, following a stroke, secondary bleeding may occur. Such bleeding, called hemorrhagic strokes.Treatment with perivascular sympathectomy and superior cervical ganglionectomy. Etiology of the disease is still unknown; however, multifactorial inheritance is considered possible because of a higher incidence of the disease in Japanese and Koreans and approximately 10% of familial occurrence among the Japanese. Recent genetic studies suggest some responsible genetic foci in chromosomes 3, 6 and 17.Keywords: Moyamoya disease, Intracranial hemorrhage, Proteomics, Stenosis
