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MRI pattern A1 (early-infantile Krabbe disease) at an advanced stage

Axial images (T2-weighted image on left, and FLAIR on right) show cystic degeneration of the pyramidal tract (white arrows), severe atrophy with enlarged insulae, and diffusely affected white matter. This patient had onset at 4 months and was 8 months of age at MRI. (Source: Krieg SI, Krägeloh-Mann I, Groeschel S, et al. Natural history of Krabbe disease: a nationwide study in Germany using clinical and MRI data. Orphanet J Rare Dis 2020;15[1]:243. Creative Commons Attribution 4.0 International [CC BY 4.0] license, creativecommons.org/licenses/by/4.0.)

Associated Disorders

  • Adult globoid cell leukodystrophy
  • Diffuse brain-sclerosis
  • Diffuse gliosis
  • Early infantile globoid cell leukodystrophy
  • Juvenile globoid cell leukodystrophy
  • Late infantile globoid cell leukodystrophy
  • Leukodystrophy