Differentiation of dys- and demyelination using diffusional anisotropy

Pediatr Neurol. 1997 Jan;16(1):63-6. doi: 10.1016/s0887-8994(96)00249-4.


We attempted differential diagnosis of dysmyelination and demyelination in childhood using magnetic resonance diffusion weighted imaging. Pelizaeus-Merzbacher disease, one of the dysmyelination disorders, demonstrated diffuse high intensity of the cerebral white matter on T2-weighted images, which demonstrated diffusional anisotropy on diffusion weighted images. On the other hand, high intensity lesions on T2-weighted images in Krabbe disease, one of the demyelination disorders, lost diffusional anisotropy. Another demyelination disorder, Alexander disease-related disorder, also lost its diffusional anisotropy. In contrast to relatively high signal of the lesions on diffusion-weighted images in Krabbe disease (high signal type), the lesions in Alexander disease-related disorder showed low signal on diffusion-weighted images (low signal type). These results suggest that diffusion-weighted images will be clinically useful to differentiate dysmyelination from demyelination; both of them demonstrate similar high intensity lesions of the white matter on T2-weighted images.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Anisotropy
  • Brain / pathology
  • Child
  • Demyelinating Diseases / diagnosis*
  • Diffuse Cerebral Sclerosis of Schilder / diagnosis
  • Diffusion
  • Female
  • Humans
  • Leukodystrophy, Globoid Cell / diagnosis
  • Magnetic Resonance Imaging*
  • Male
  • Nerve Fibers, Myelinated / pathology