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Related Experiment Videos

Alexander disease: diagnosis with MR imaging.

M S van der Knaap1, S Naidu, S N Breiter

  • 1Department of Child Neurology, Free University Hospital, Amsterdam, the Netherlands.

AJNR. American Journal of Neuroradiology
|March 10, 2001
PubMed
Summary

Magnetic resonance imaging (MRI) criteria can now diagnose Alexander disease in vivo. This reduces the need for brain biopsies, except in rare, atypical cases.

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Area of Science:

  • Neurology
  • Radiology
  • Medical Diagnostics

Background:

  • Alexander disease diagnosis traditionally requires identifying Rosenthal fibers via brain biopsy or autopsy.
  • Magnetic resonance imaging (MRI) offers a potential non-invasive diagnostic alternative.

Purpose of the Study:

  • To establish reliable MRI criteria for diagnosing Alexander disease.
  • To assess the feasibility of an MRI-based diagnosis, reducing reliance on invasive procedures.

Main Methods:

  • Analyzed MRI scans from three autopsy-confirmed Alexander disease cases to define diagnostic criteria.
  • Applied these criteria retrospectively to 217 pediatric leukoencephalopathy cases.

Main Results:

  • Defined five key MRI criteria, including specific white matter changes, signal abnormalities, and contrast enhancement patterns.

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  • Identified 19 patients meeting these criteria, with four subsequently confirmed histologically.
  • Observed strong correlation between MRI findings, clinical symptoms, and known histopathology.

    • Conclusions:

    • Established MRI criteria enable accurate in vivo diagnosis of Alexander disease.
    • Brain biopsy is now only necessary for atypical presentations, significantly advancing diagnostic capabilities.