Recent Advances in Moyamoya Disease: Pathophysiology and Treatment
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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.
KeywordsMoyamoya Latest advancements Pathophysiology Genetics Revascularization Direct bypass Cerebral perfusion studies Neuroimaging Outcome Cognition
Cerebral blood flow
Digital subtraction angiography
Endothelial progenitor cell
Internal carotid artery
Middle cerebral artery
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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