Recent Advances in Moyamoya Disease: Pathophysiology and Treatment

  • Annick Kronenburg
  • Kees P. J. Braun
  • Albert van der Zwan
  • Catharina J. M. KlijnEmail author
Stroke (H Adams, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Stroke


Moyamoya disease is a progressive intracranial arteriopathy characterized by bilateral stenosis of the distal portion of the internal carotid artery and the proximal anterior and middle cerebral arteries, resulting in transient ischemic attacks or strokes. The pathogenesis of moyamoya disease remains unresolved, but recent advances have suggested exciting new insights into a genetic contribution as well as into other pathophysiological mechanisms. Treatment that may halt progression of the disease or even reverse the intracranial arteriopathy is yet to be found. There are strong indications that neurosurgical intervention, through direct, indirect, or combined revascularization surgery, can reduce the risk of ischemic stroke and possibly also cognitive dysfunction by improving cerebral perfusion, although randomized clinical trials have not been performed. Many questions regarding the indication for and timing of surgery remain unanswered. In this review, we discuss recent developments in the pathogenesis and treatment of moyamoya disease.


Moyamoya Latest advancements Pathophysiology Genetics Revascularization Direct bypass Cerebral perfusion studies Neuroimaging Outcome Cognition 



Cerebral blood flow


Computed tomography


Cerebrovascular reserve


Digital subtraction angiography


Endothelial progenitor cell


Internal carotid artery


Middle cerebral artery


Moyamoya disease


Matrix metalloproteinase


Moyamoya syndrome


Moyamoya vasculopathy


Magnetic resonance imaging


Positron emission tomography


Randomized controlled trial


Smooth muscle cell


Superficial temporal artery


Transient ischemic attack


Compliance with Ethics Guidelines

Conflict of Interest

Annick Kronenburg, Kees P.J. Braun, and Albert van der Zwan declare that they have no conflict of interest.

Catharina J.M. Klijn has grants pending from the Dutch Brain Foundation [2012(1)-179], the Tutein Nolthenius Oldenhof Fund, and the Dutch Heart Foundation (Clinical Established Investigator grant 2012 T077).

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.


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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Annick Kronenburg
    • 1
  • Kees P. J. Braun
    • 1
  • Albert van der Zwan
    • 1
  • Catharina J. M. Klijn
    • 1
    Email author
  1. 1.Department of Neurology and NeurosurgeryBrain Center Rudolf Magnus, UMC UtrechtUtrechtThe Netherlands

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