Moyamoya disease in Korea

Area of Science:

• Neurology
• Pediatric Neurology
• Vascular Neurology

Background:

• Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal internal carotid arteries and the development of collateral vessels.
• Understanding the distinct clinical presentations and imaging findings in pediatric versus adult populations is crucial for accurate diagnosis and management.

Purpose of the Study:

• To analyze and compare the clinical manifestations, computed tomography (CT), and angiographic findings of moyamoya disease in children and adults.
• To identify differences in neurological deficits and lesion patterns between pediatric and adult patients with moyamoya disease.

Main Methods:

• Retrospective analysis of 38 moyamoya disease cases (21 children, 17 adults) over 16 years at Yonsei University Medical Center.
• Review of clinical data, including chief complaints and neurological deficits (transient and permanent).
• Evaluation of computed tomography (CT) and cerebral angiography findings, focusing on lesion location and type (infarction vs. hemorrhage).

Main Results:

• The majority of moyamoya disease attacks occurred in spring for both children and adults.
• Children most commonly presented with hemiparesis and convulsions, progressing to hemiplegia, with CT showing predominantly infarction (90.6%).
• Adults most commonly presented with loss of consciousness and headache, with cerebral angiography revealing bilateral carotid artery occlusion and CT showing hemorrhage (88.2%).

Conclusions:

• Moyamoya disease exhibits distinct clinical and radiological profiles in pediatric and adult populations.
• Children are more prone to ischemic events like infarction and hemiplegia, whereas adults are more susceptible to hemorrhagic events.
• Bilateral carotid artery occlusion is the predominant angiographic finding in both age groups, highlighting the shared underlying pathology.