Moyamoya Disease

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Background

Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by progressive
stenosis of the arteries of the circle of Willis, and formation of a collateral vascular network
at the base of the brain. Moyamoya syndrome is caused by numerous conditions, which
lead to arterial occlusion of the circle of Willis and resultant collaterals reminiscent
of moyamoya disease. Due to the diverse angioarchitecture and unknown influence factors, the
hemodynamic disturbances of MMD/Moyamoya syndrome are complicated, and result in
multimodal clinical manifestations including headache, cognitive impairment, seizures, TIA,
infarction, and hemorrhage. Surgical revascularization has been proven as effective in
decreasing incidence of long-term risk of stroke. However, occurrence of perioperative stroke
and complications such as seizures and cerebral hyperperfusion syndrome are still
unpredictable. In addition, many patients with cognitive impairment cannot benefit from
surgical revascularization and the mechanism remains unclear. Thus, a series of studies focusing
on the objective evaluation of MMD/Moyamoya syndrome are needed to help understand this
complicated cerebrovascular disease and improve our treatment strategy.

The goal of this Research Topic is to better understand the pathophysiological nature of
MMD/Moyamoya syndrome through objective neuroimaging. We also aim to generate a
reasonable and objective evaluation system for MMD/ Moyamoya syndrome and would like
to understand the link between cognitive impairment and brain damage of
MMD/Moyamoya syndrome though neuroimaging. Through these findings we also aim to
improve the treatment strategy by understanding the pathophysiological link with relevant
complications. Finally, we also aim to improve our understanding of the significance of
perioperative management for patients with severe complications from the perspective of
objective evaluation.

For this Research Topic we welcome manuscripts ranging from providing objective
neuropsychological and neuroimaging evaluations of MMD/Moyamoya syndrome, to those
investigating the pathophysiological basis of MMD/ Moyamoya syndrome. Studies both in
adults and pediatric patients are welcome, however studies describing long-term outcomes
of MMD/Moyamoya syndrome with no relevance to objective evaluations will not be
considered. Examples of topics we would like to cover in this collection are:

• Genetic studies that relate biological features to the angioarchitecture, hemodynamics, or
clinical manifestations of MMD/Moyamoya syndrome;
• Neuropsychological nature of MMD/Moyamoya syndrome and novel evaluation tools;
• Neuronal interaction and network models of MMD/ Moyamoya syndrome;
• The relationship between treatment strategy and relevant complications;
• The relationship between perioperative management and relevant complications;
• Reviews of recent advancements in the objective evaluation of MMD/ Moyamoya
syndrome.

Keywords: moyamoya disease, neuroimaging, evaluation

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