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Computed tomography in Alexander's disease.

K Farrell, S Chuang, L E Becker

    Annals of Neurology
    |June 1, 1984
    PubMed
    Summary
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    Computed tomography revealed specific contrast-enhancing lesions in infants with Alexander's disease. These findings aid in distinguishing it from Canavan's disease, potentially avoiding brain biopsies.

    Area of Science:

    • Neurology
    • Radiology
    • Pediatric Neurology

    Background:

    • Alexander's disease is a rare genetic leukoencephalopathy.
    • Distinguishing Alexander's disease from other white matter disorders can be challenging.
    • Diagnostic brain biopsy is often invasive and carries risks.

    Observation:

    • Computed tomography (CT) imaging in an infant with Alexander's disease revealed characteristic contrast-enhancing lesions.
    • These lesions were located in the periventricular frontal regions, caudate nuclei, and thalami.
    • The distribution of enhancement correlated with areas of prominent Rosenthal fibers.

    Findings:

    • The observed pattern of contrast enhancement is unique to Alexander's disease.
    • This radiological signature differs from patterns seen in other white matter diseases.

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  • Rosenthal fibers, a hallmark of Alexander's disease, appear to influence lesion localization.

    • Implications:

    • These CT findings offer a non-invasive method for diagnosing Alexander's disease.
    • This may reduce the need for diagnostic brain biopsies in affected infants.
    • Improved diagnostic accuracy can lead to earlier management and better patient outcomes.