Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis).

Neurologia medico-chirurgica, 2012

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The characteristics of moyamoya disease (spontaneous occlusion of the circle of Willis, cerebrovascular ``moyamoya'' disease) on cerebral angiography were reported for the first time in 1957,8) and the concept of moyamoya disease as a separate disease entity was established in the 1960s.1–3,6,7) Pathologically, moyamoya disease is characterized by chronic progressive stenosis of the terminal portion of the bilateral internal carotid arteries, which leads to the formation of an abnormal vascular network composed of collateral pathways at the base of the brain (moyamoya vessels at the base of the brain) (`moyamoya' is the Japanese term for a ``puff of smoke,'' which has been used to describe the appearance of these collateral vessels on cerebral angiograms6)). Eventually, with bilateral internal carotid artery occlusion, the moyamoya vessels at the base of the brain derived from the internal carotid arteries disappear, and the entire brain is perfused by the external carotid artery system and the vertebrobasilar artery system.1–3,5–7) This disease is included in the list of diseases for Research on Measures for Intractable Diseases and the Specified Disease Treatment Research Program specified by the Ministry of Health, Labour and Welfare. Currently, the diagnostic criteria for moyamoya disease (spontaneous occlusion of the circle of Willis) laid down by the research committee are as follows.4)

Subfields: Neurology, Psychiatry and Mental health, Rheumatology

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