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Cerebrovascular Moyamoya disease.

Y Yamashiro, H Takahashi, K Takahashi

    European Journal of Pediatrics
    |April 1, 1984
    PubMed
    Summary

    Moyamaya disease in children often presents with headaches or motor deficits. Electroencephalography showing delta bursts after hyperventilation may indicate this cerebrovascular condition.

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    Area of Science:

    • Neurology
    • Pediatrics
    • Vascular Medicine

    Background:

    • Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries.
    • Understanding its clinical presentation and diagnostic features in children is crucial for timely intervention.

    Observation:

    • Ten Japanese pediatric cases of Moyamoya disease were analyzed, alongside a review of international literature.
    • Common initial symptoms included headache (40%) and motor deficits/convulsions (30%).
    • Recurrent symptoms frequently involved motor deficits (80%) and headaches (40%).

    Findings:

    • The clinical presentation in Japanese children mirrored international cases, with a notable female predominance.
    • Electroencephalography (EEG) revealed characteristic high-voltage delta bursts post-hyperventilation in all affected children.
    • Vascular imaging identified stenosis/occlusion in the terminal carotid arteries and basal ganglia collateral networks.

    Implications:

    • The study highlights the utility of EEG with hyperventilation in suspecting Moyamoya disease in children presenting with neurological deficits.
    • Early suspicion and diagnosis can lead to prompt management of this progressive cerebrovascular condition.
    • Further research into the specific EEG patterns associated with Moyamoya disease in pediatric populations is warranted.

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