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Acta Neuropathologica

, Volume 40, Issue 3, pp 213–218 | Cite as

Generalized giant axonal neuropathy

A filament-forming disease of neuronal, endothelial, glial, and schwann cells in a patient without kinky hair
  • Jürgen Peiffer
  • Wolfgang Schlote
  • Albert Bischoff
  • Eugen Boltshauser
  • Günter Müller
Original Investigations

Summary

The process of Giant Axonal Neuropathy (GAN) is not restricted to the peripheral nerves, but also involves the central nervous system. In a 25 year old man with normal hair, abundant axon swellings and spheroids were observed in the spinal cord, brain system, and cerebral cortex. The findings in the sural nerve have already been published by Boltshauser et al. (1977). Accumulations of filaments in the axons and in the perineural cells were accompanied by Rosenthal fibres. The ultrastructural pattern of GAN differs clearly from that of Neuroaxonal Dystrophies.

Key words

Giant axonal neuropathy Neuroaxonal dystrophies Kinky hair Rosenthal fibres Neurofilaments 

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Copyright information

© Springer-Verlag 1977

Authors and Affiliations

  • Jürgen Peiffer
    • 1
  • Wolfgang Schlote
    • 2
  • Albert Bischoff
    • 3
  • Eugen Boltshauser
    • 4
  • Günter Müller
    • 5
  1. 1.Institute of Brain ResearchUniversity of TübingenTübingenGermany
  2. 2.Department of Submicroscopic Pathology and NeuropathologyUniversity of TübingenTübingenGermany
  3. 3.Department of NeurologyUniversity of CH-3000 BernSwitzerland
  4. 4.Department of PediatricsUniversity of CH-8000 ZürichSwitzerland
  5. 5.Department of PathologyKantonspital CH-7000 ChurSwitzerland

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