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Pseudomoyamoya in sickle cell anemia

R Garza-Mercado

    Surgical Neurology
    |December 1, 1982
    PubMed
    Summary

    Sickle cell anemia, a rare hereditary blood disorder, can lead to cerebrovascular accidents. This case highlights a moyamoya-like pattern in a patient with sickle cell anemia, suggesting a pseudomoyamoya diagnosis.

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    Area of Science:

    • Neurology
    • Hematology
    • Genetics

    Background:

    • Sickle cell anemia is a rare hereditary blood disorder.
    • Cerebrovascular accidents are a known complication of sickle cell anemia.
    • Moyamoya disease is a rare cerebrovascular disorder of unknown etiology.

    Observation:

    • A 13-year-old Mexican boy with sickle cell anemia presented with a cerebrovascular accident.
    • Computerized tomography (CT) confirmed a brain stem stroke.
    • Angiography revealed a moyamoya-like pattern in cerebral vasculature.

    Findings:

    • The patient exhibited a moyamoya-like angiographic pattern secondary to sickle cell anemia.
    • This presentation differs from idiopathic moyamoya disease due to the known underlying condition.
    • The findings support the classification of this condition as pseudomoyamoya.

    Implications:

    • This case expands the understanding of cerebrovascular complications in sickle cell anemia.
    • Distinguishing pseudomoyamoya from true moyamoya is crucial for accurate diagnosis and management.
    • Further research is needed to elucidate the pathogenesis of pseudomoyamoya in sickle cell disease.

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