J Korean Med Assoc.  2019 Nov;62(11):577-585. 10.5124/jkma.2019.62.11.577.

Diagnosis and treatment of adult Moyamoya disease

Affiliations
  • 1Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Korea. eunkim@snu.ac.kr

Abstract

Moyamoya disease (MMD) refers to a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery with abnormal collateral vessel formation of unknown etiology. The definite diagnosis of MMD requires cerebral angiography or magnetic resonance angiography and/or magnetic resonance imaging after excluding other underlying diseases, particularly in adult patients. The treatment aims to improve regional cerebral blood flow to prevent cerebral ischemic events and alleviate hemodynamic instability that can provoke cerebral hemorrhage. Although various surgical revascularization methods have been introduced, combined revascularization surgery including direct revascularization is preferred over indirect revascularization only in adult MMD patients. Several recent studies have shown that surgical treatment has better outcomes and prognosis for symptomatic hemodynamically unstable MMD patients with both ischemic and hemorrhagic presentations. For asymptomatic patients, follow up with appropriate imaging is recommended. Surgery should be considered when new symptoms emerge with hemodynamic aggravation.

Keyword

Diagnosis; Therapy; Adult; Moyamoya disease

MeSH Terms

Adult*
Carotid Artery, Internal
Cerebral Angiography
Cerebral Hemorrhage
Cerebrovascular Circulation
Diagnosis*
Follow-Up Studies
Hemodynamics
Humans
Magnetic Resonance Angiography
Magnetic Resonance Imaging
Moyamoya Disease*
Prognosis
Full Text Links
  • JKMA
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2022 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr