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Computed tomography in Wilson disease

S I Harik, M J Post

    Neurology
    |January 1, 1981
    PubMed
    Summary
    This summary is machine-generated.

    Computed tomography (CT) reveals low-density abnormalities in the basal ganglia and brainstem for cerebral Wilson disease patients. These CT findings in Wilson disease may not always correlate with clinical status or treatment response.

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    Area of Science:

    • Neurology
    • Radiology
    • Medical Imaging

    Background:

    • Wilson disease is a genetic disorder causing copper accumulation.
    • Cerebral and hepatic forms manifest distinct clinical symptoms.
    • Kayser-Fleischer rings are a hallmark of Wilson disease.

    Purpose of the Study:

    • To evaluate the utility of computed tomography (CT) in diagnosing and monitoring Wilson disease.
    • To characterize CT findings in different forms of Wilson disease.
    • To assess the correlation between CT abnormalities and clinical status.

    Main Methods:

    • Computed tomography (CT) scans were performed on six patients diagnosed with Wilson disease.
    • Five patients presented with the cerebral form, and one with the hepatic form.

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  • CT scans were analyzed for abnormalities, including density changes and contrast enhancement patterns.

    • Main Results:

    • All five patients with cerebral Wilson disease exhibited CT abnormalities, predominantly low-density lesions.
    • Abnormalities were consistently found in the basal ganglia, with some cases showing cerebellar involvement.
    • The single patient with hepatic Wilson disease showed no CT abnormalities.
    • CT findings did not always correlate with clinical status, and abnormalities sometimes worsened despite treatment.

    Conclusions:

    • CT can detect characteristic brain abnormalities in cerebral Wilson disease, primarily affecting the basal ganglia.
    • CT findings in Wilson disease may not reliably reflect the clinical condition or treatment efficacy.
    • CT is not indicated for the hepatic form of Wilson disease due to a lack of abnormalities.