Metachromatic leukodystrophy: Comparison of early-and late-onset forms

Academic Article

Abstract

  • Comparative study of peripheral nerve biopsies and cultured skin fibroblasts from patients with late infantile and early adult-onset forms of metachromatic leukodystrophy revealed similar pathologic changes in the early stages of nerve degeneration. However, the peripheral nerves in the adult-onset cases eventually reached a more chronically demyelinated fibrotic state than did nerves in the infantile cases. Cultured skin fibroblasts from the adult-onset patients, although clearly abnormal, were able to catabolize sulfatide significantly more effectively than the cultured skin fibroblasts from late infantile patients. © 1977 American Academy of Neurology.
  • Authors

    Published In

  • Neurology  Journal
  • Digital Object Identifier (doi)

    Author List

  • Percy AK; Kaback MM; Herndon RM
  • Start Page

  • 933
  • End Page

  • 941
  • Volume

  • 27
  • Issue

  • 10