Presumed immune-mediated cerebellar granuloprival degeneration in the Coton de Tulear breed

A. Tipold, R. Fatzer, A. Jaggy, P. Moore, M. Vandevelde

Research output: Contribution to journalArticle

21 Scopus citations

Abstract

An unusual form of cerebellar granuloprival degeneration was observed in three male Coton de Tulear puppies between 12 and 14 weeks of age from different litters showing progressive cerebellar signs beginning at 8 weeks after birth. Pathological examinations revealed a shrunken cerebellum. Histopathologically the granular cells were diminished or almost completely absent, some 'torpedos' of Purkinje cells were present. There was a marked gliosis, and occasionally small inflammatory foci were present. A marked diffuse T cell infiltration (CD3+ cells) occurred in the lesions, B cells did not appear. CD18 staining showed an upregulation of microglial cells at the lesion site. Histopathologically the lesions resembled paraneoplastic cerebellar degeneration which is caused by an autoimmune mediated T cell reaction. This congenital condition in the Coton de Tulear dog breed could be based on a genetically defined immune defect leading to autoimmune destruction of the granular cells. (C) 2000 Elsevier Science B.V.

Original languageEnglish (US)
Pages (from-to)130-133
Number of pages4
JournalJournal of Neuroimmunology
Volume110
Issue number1-2
DOIs
StatePublished - Oct 2 2000

Keywords

  • Cerebellar granuloprival degeneration
  • Coton de Tulear breed
  • Microglia
  • T cells

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy
  • Clinical Neurology
  • Neurology

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