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REVIEW article

Front. Hum. Neurosci.
Sec. Brain Health and Clinical Neuroscience
Volume 19 - 2025 | doi: 10.3389/fnhum.2025.1503639
This article is part of the Research Topic Alterations in brain structure, function and cognitive abilities caused by cerebrovascular diseases View all 5 articles

Research progress in unilateral moyamoya disease

Provisionally accepted
Xiaokuan Hao Xiaokuan Hao 1*Cunxin Tan Cunxin Tan 2*Ziqi Liu Ziqi Liu 1Yang Tie Yang Tie 1*Yanru Wang Yanru Wang 1Shihao He Shihao He 3Ran Duan Ran Duan 2*Rong Wang Rong Wang 4,5*
  • 1 Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China
  • 2 Department of Neurosurgery, International Hospital, Peking University, Beijing, Beijing Municipality, China
  • 3 Department of Neurosurgery, Peking Union Medical College Hospital (CAMS), Beijing, Beijing Municipality, China
  • 4 Beijing Tiantan Hospital, Capital Medical University, Beijing, China
  • 5 National Clinical Research Center for Neurological Diseases (China), Beijing, Beijing, China

The final, formatted version of the article will be published soon.

    Unilateral moyamoya disease (U-MMD) is a chronic vascular disease characterized by progressive stenosis and occlusion of the terminal end of the internal carotid artery and its main branches, resulting in the appearance of moyamoya-like blood vessels at the base of the brain. The etiology of U-MMD is unknown, it accounts for 9.7-17.8% of all moyamoya disease, and the family incidence is 5.5-13.3%. The clinical characteristics are similar to those of typical moyamoya disease, but there are some differences. U-MMD can progress to bilateral moyamoya disease with a median probability of 29.01% (ranging from 6.3% to 58.8%), and there are many risk factors that promote its development. Surgical treatment can effectively reduce the incidence of ischemic stroke and improve prognosis. However, the timing and indications for surgery require further investigation. This article reviews the latest research progress on the etiology, epidemiology, clinical and radiological characteristics, progression, treatment, and prognosis of U-MMD.

    Keywords: Unilateral moyamoya disease, etiology, Epidemiology, Clinical course, progression, Surgery

    Received: 29 Sep 2024; Accepted: 13 Jan 2025.

    Copyright: © 2025 Hao, Tan, Liu, Tie, Wang, He, Duan and Wang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

    * Correspondence:
    Xiaokuan Hao, Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China
    Cunxin Tan, Department of Neurosurgery, International Hospital, Peking University, Beijing, 100871, Beijing Municipality, China
    Yang Tie, Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100050, China
    Ran Duan, Department of Neurosurgery, International Hospital, Peking University, Beijing, 100871, Beijing Municipality, China
    Rong Wang, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

    Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.