Recent progress in hemorrhagic moyamoya disease.

Area of Science:

• Neurology
• Vascular Neurology
• Neuroimaging

Background:

• Moyamoya disease (MMD) is a progressive cerebrovascular disorder with ischemic, hemorrhagic, and asymptomatic presentations.
• Hemorrhagic MMD is notable for distinct imaging findings and debated treatment strategies.

Purpose of the Study:

• To comprehensively review the literature on hemorrhagic Moyamoya disease.
• To focus on epidemiology, etiology, pathogenesis, imaging, hemorrhage predictors, and treatment efficacy.

Main Methods:

• Systematic literature review of hemorrhagic Moyamoya disease.
• Analysis of epidemiological data, etiological factors, and pathogenetic mechanisms.
• Evaluation of diagnostic imaging features and predictive markers for hemorrhage.

Main Results:

• Hemorrhagic MMD predominantly affects adult populations in Asian countries.
• Key imaging features include abnormal anterior choroidal/posterior communicating artery branching and microbleeds.
• Current treatment protocols lack standardization, necessitating further research.

Conclusions:

• Hemorrhagic MMD requires further investigation into standardized treatment approaches.
• Prospective randomized trials are crucial for determining optimal therapeutic strategies.
• Cognitive function and quality of life are critical outcomes in evaluating treatment effectiveness.