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MRI pattern B1: MRI in later-onset Krabbe disease

White matter is primarily affected in the periventricular parieto-occipital regions (arrows in the upper right FLAIR image, and lower left sagittal and lower right T2-weighted images). The FLAIR image shows involvement of the splenium of the corpus callosum (black arrow). Cerebellar white matter is not affected (axial T2-weighted image upper left). This patient had onset at 5 years, and was 5 years 1 month 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.)

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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