Acta Neuropathologica

, Volume 97, Issue 2, pp 201–207

Nuclear inclusions in spinocerebellar ataxia type 1

  • C. Duyckaerts
  • A. Dürr
  • G. Cancel
  • A. Brice
Case report

DOI: 10.1007/s004010050975

Cite this article as:
Duyckaerts, C., Dürr, A., Cancel, G. et al. Acta Neuropathol (1999) 97: 201. doi:10.1007/s004010050975

Abstract

Spinocerebellar ataxia type 1 is due to a CAG repeat expansion in the gene encoding ataxin-1. In a case with an expansion of 56 repeats, intranuclear inclusions were found only in neurons, both in severely affected regions (such as the pons) and in areas where the lesions were inconspicuous (such as the cortex or the striatum). The inclusions were labelled by a monoclonal antibody directed against long polyglutamine stretches (1C2); they were also detected by the anti-ubiquitin antibody. They were faintly eosinophilic, Congo red negative and were not stained by thioflavin S or by ethidium bromide.

Copyright information

© Springer-Verlag Berlin Heidelberg 1999

Authors and Affiliations

  • C. Duyckaerts
    • 1
  • A. Dürr
    • 2
  • G. Cancel
    • 2
  • A. Brice
    • 2
  1. 1.Laboratoire de Neuropathologie Escourolle, INSERM U 360, Association Claude Bernard, Hôpital de la Salpêtrière, 47 Blvd de l’Hôpital, F-75651 Paris Cedex 13, France e-mail: charles.duyckaerts@psl.ap-hop-paris.fr, Fax: +33-1-44-23-98-28FR
  2. 2.INSERM U 289, Hôpital de la Salpêtrière, Paris, FranceFR