Acta Neuropathologica

, Volume 98, Issue 3, pp 304–308

Evidence of widespread axonal pathology in Wolfram syndrome

  • P. Shannon
  • L. Becker
  • J. Deck
Case report

DOI: 10.1007/s004010051084

Cite this article as:
Shannon, P., Becker, L. & Deck, J. Acta Neuropathol (1999) 98: 304. doi:10.1007/s004010051084

Abstract

Wolfram syndrome, characterised by diabetes insipidus, diabetes mellitus, optic atrophy sensorineural deafness and acquired urinary tract abnormalities, is an hereditary neurodegenerative syndrome, the pathogenesis of which is unknown. We report the post-mortem findings on a patient with well-documented Wolfram syndrome. The brain showed severe degeneration of the optic nerves, chiasm and tracts as well as severe loss of neurons from the lateral geniculate nuclei, basis pontis, and the hypothalamic paraventricular and supraoptic nuclei. In addition, there was a widespread axonal dystrophy with axonal swellings in the pontocerebellar tracts, the optic radiations, the hippocampal fornices and the deep cerebral white matter. This widespread axonal pathology parallels the pattern of neurodegeneration and in many areas is more striking than neuronal loss.

Key words Wolfram syndromePathologyAxonaldystrophy

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • P. Shannon
    • 1
  • L. Becker
    • 2
  • J. Deck
    • 3
  1. 1.Division of Neuropathology, Department of Pathology and Laboratory Medicine, The Toronto Hospital, Western Division, and the University of Toronto, Department of Pathobiology and Laboratory Medicine, 399 Bathurst Street, Toronto, Ontario, Canada M5T 2SB Tel.: +1-416-603-5722, Fax: +1-416-813-5974CA
  2. 2.Department of Pediatric Laboratory Medicine, The Hospital for Sick Children, and Department of Pathobiology and Laboratory Medicine, The University of Toronto, Toronto, Ontario, CanadaCA
  3. 3.Office of the Chief Coroner of Ontario, Forensic Pathology Unit, Department of Pathobiology and Laboratory Medicine, The University of Toronto, Toronto, Ontario, CanadaCA