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Neuropediatrics 1974; 5(2): 191-209
DOI: 10.1055/s-0028-1091702
Case report

© 1974 by Thieme Medical Publishers, Inc.

Infantile Globoid Cell Leucodystrophy (Krabbe's Disease) – Some remarks on clinical, biochemical and sural nerve biopsy findings

E. M. G. Joosten1 3 , J. B. Krijgsman2 , A. A. W. M. Gabreëls-Festen3 , F. J. M. Gabreëls3 , P. E. C. Baars2
  • 1Submicroscopic Morphology, University of Nijmegen, The Netherlands
  • 2Department of Paediatrics, University of Nijmegen, The Netherlands
  • 3Department of Neurology, University of Nijmegen, The Netherlands
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1974

1974

Publication Date:
18 November 2008 (online)

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This paper discusses the pathological findings in a sural nerve biopsy. There was evidence of a relation between myelin breakdown and storage material. No evidence was found of a primary axonal degeneration; only segmental de- and remyelinisation. The diminished density of myelinated fibers was only secondary to an increase in endoneurium.

Globoid cell leucodystrophy - sural nerve biopsy