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Wilson's disease. Clinical correction with cranial computed tomography

B E Kendall, S S Pollock, N M Bass

    Neuroradiology
    |January 1, 1981
    PubMed
    Summary

    Computed tomography (CT) reveals brain abnormalities in Wilson's disease patients with neurological symptoms. Basal ganglia lesions on CT correlate with extrapyramidal dysfunction, aiding disease management.

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    Area of Science:

    • Neurology
    • Radiology
    • Medical Imaging

    Background:

    • Wilson's disease is a genetic disorder causing copper accumulation.
    • Neurological manifestations are common and can be severe.
    • Imaging techniques are crucial for diagnosis and monitoring.

    Purpose of the Study:

    • To correlate cranial computed tomogram (CT) findings with clinical symptoms in Wilson's disease patients.
    • To assess the relationship between CT abnormalities and specific neurological deficits.
    • To evaluate the utility of CT in managing Wilson's disease.

    Main Methods:

    • Cranial computed tomograms (CT) were performed on 12 patients with confirmed Wilson's disease.
    • CT findings were correlated with clinical assessments of neurological disturbances.

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  • Specific attention was given to basal ganglia, cerebral, and cerebellar structures.

    • Main Results:

    • CT abnormalities were present in 8/12 patients, all with neurological symptoms.
    • Basal ganglia atrophy and low-density lesions on CT correlated significantly with extrapyramidal dysfunction.
    • Cerebral and cerebellar atrophy showed moderate correlation with intellectual dysfunction and ataxia, respectively.
    • No specific CT signs were identified for brainstem involvement.
    • CT scans showed marked abnormalities within a year of symptom onset; most severe scans were in patients with longer untreated disease duration.

    Conclusions:

    • CT findings, particularly basal ganglia abnormalities, correlate well with neurological deficits in Wilson's disease.
    • CT can aid in monitoring disease progression and response to treatment (cupruresis).
    • CT is a valuable tool in the comprehensive management of Wilson's disease.