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Recent Advances in Moyamoya Disease: Pathophysiology and Treatment

  • Stroke (H Adams, Section Editor)
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Abstract

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.

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Abbreviations

CBF:

Cerebral blood flow

CT:

Computed tomography

CVR:

Cerebrovascular reserve

DSA:

Digital subtraction angiography

EPC:

Endothelial progenitor cell

ICA:

Internal carotid artery

MCA:

Middle cerebral artery

MMD:

Moyamoya disease

MMP:

Matrix metalloproteinase

MMS:

Moyamoya syndrome

MMV:

Moyamoya vasculopathy

MRI:

Magnetic resonance imaging

PET:

Positron emission tomography

RCT:

Randomized controlled trial

SMC:

Smooth muscle cell

STA:

Superficial temporal artery

TIA:

Transient ischemic attack

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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).

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

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  1. Department of Neurology and Neurosurgery, Brain Center Rudolf Magnus, UMC Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands

    Annick Kronenburg, Kees P. J. Braun, Albert van der Zwan & Catharina J. M. Klijn

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  1. Annick Kronenburg
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  2. Kees P. J. Braun
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Kronenburg, A., Braun, K.P.J., van der Zwan, A. et al. Recent Advances in Moyamoya Disease: Pathophysiology and Treatment. Curr Neurol Neurosci Rep 14, 423 (2014). https://doi.org/10.1007/s11910-013-0423-7

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