2021 Japanese Guidelines for the Management of Moyamoya Disease: Guidelines from the Research Committee on Moyamoya Disease and Japan Stroke Society

• Published in: Neurologia Medico-Chirurgica Vol. 62; no. 4; pp. 165 - 170
• Main Authors: FUJIMURA, Miki, TOMINAGA, Teiji, KURODA, Satoshi, TAKAHASHI, Jun C., ENDO, Hidenori, OGASAWARA, Kuniaki, MIYAMOTO, Susumu, Research Committee on Moyamoya Disease (Spontaneous Occlusion of Circle of Willis) of the Ministry of Health, Labor Welfare, Japan, the Guideline Committee 2021 of the Japan Stroke Society
• Format: Journal Article
• Language: English
• Published: Japan The Japan Neurosurgical Society 15.04.2022; THE JAPAN NEUROSURGICAL SOCIETY; Japan Science and Technology Agency
• Subjects: Cerebral Revascularization; guideline; Humans; Japan; management; Moyamoya disease; Moyamoya Disease - diagnosis; Moyamoya Disease - therapy; revascularization surgery; Special Topic; Stroke - diagnosis; Stroke - etiology; Stroke - therapy; Treatment Outcome; Japan; revascularization surgery; management; guideline; moyamoya disease
• ISSN: 0470-8105; 1349-8029
• DOI: 10.2176/jns-nmc.2021-0382

[Introduction] The angiographic characteristics of moyamoya disease (MMD) were first reported in 1957 as hypogenesis of bilateral internal carotid arteries (ICAs). Then, the concept of MMD was established as an independent disease entity in the 1960s. MMD is characterized by chronic progressive stenosis of the terminal portion of the bilateral ICAs, which leads to the formation of an abnormal vascular network, functioning as a collateral pathway at the base of the brain. The appearance of this vascular network on cerebral angiography was similar to a "puff of smoke," which was described as "moyamoya" in Japanese. As the steno-occlusive changes of the bilateral ICAs progress, the moyamoya vessels eventually regress, and the entire cerebral hemisphere is perfused by the external carotid artery and the vertebrobasilar artery systems. MMD was designated as an intractable disease in Japan, which was initiated by the Intractable Disease Health Care Act to secure a budget for maintaining medical expense subsidy for patients with intractable diseases and to promote research on clarifying the pathogenesis.