Moyamoya disease in children.

Area of Science:

• Neurology
• Vascular Medicine
• Radiology

Background:

• Moyamoya disease is a rare cerebrovascular condition.
• It features progressive steno-occlusive changes in terminal internal carotid arteries and associated moyamoya vessels.
• The disease has a high incidence in East Asia, with familial cases comprising 10-15%.

Purpose of the Study:

• To summarize the characteristics and diagnostic approaches for Moyamoya disease.
• To highlight the utility of non-invasive imaging techniques.

Main Methods:

• Review of literature on Moyamoya disease.
• Discussion of diagnostic criteria and imaging findings.
• Emphasis on Magnetic Resonance Imaging (MRI) and MR angiography (MRA).

Main Results:

• Moyamoya disease presents with two peaks in age distribution (around 5 and 40 years).
• Pediatric patients commonly experience transient ischemic attacks, infarction, headache, and involuntary movements.
• Typical MRI/MRA findings include steno-occlusive changes in the internal carotid arteries and abnormal basal ganglia vasculature.

Conclusions:

• MRI and MRA are valuable non-invasive tools for diagnosing and monitoring Moyamoya disease.
• Cerebral angiography, while the gold standard, may not be mandatory if MRI/MRA findings are typical.
• Specific MRI findings like T2 shortening, ivy sign, and medullary streaks further support diagnosis.