No medical treatment has been used to prevent progression of moyamoya disease. Bypass surgery is considered to be effective for improving cerebral blood flow and metabolism and preventing progression. The incidence of ischemic attack rapidly decreases after bypass surgery and reblee ding might also be reduced. Moyamoya vessels start to regress after bypass surgery, and the deep temporal artery and the middle meningeal artery increase their calibers and can be identified by MRA [29] . Stenotic change in the carotid terminal quickly progress after surgery [30] .
Conclusions
Regardless of the course, moyamoya disease inevitably progresses in the majority of patients. Once a patient manifests the progression of cerebrovascular occlusive lesion or ischemic symptoms, bypass surgery should be performed for the affected hemisphere after the confirmation of the flow compromise.
124 K. Hayashi and I. Nagata
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Introduction
Moyamoya disease is a rare cerebrovascular occlusive disorder that is characterized by stenosis or occlusion of the distal internal carotid or proximal anterior or middle cerebral arteries, which causes the formation of multiple tiny collateral vascular networks (moyamoya vessels) at the base of the brain [1– 3] . While this disorder commonly occurs alone (moyamoya disease), it occasionally occurs with well-recognized associated conditions including sickle cell disease, neurofibromatosis type I, cranial therapeutic irradiation, and Down syndrome (moyamoya syndrome) [4] .
In addition to typical intracranial vascular lesions associated with moyamoya disease, steno-occlusive lesions of extracranial vessels including renal, coronary, pulmonary, mesenteric, and peripheral arteries have also been reported [5– 31] . Among these extracranial arteries, moyamoya disease most commonly impacts the renal artery [12– 31] .