This study was performed at the University of Veterinary Medicine Vienna, Vienna, Austria.
Spongy Degeneration with Cerebellar Ataxia in Malinois Puppies: A Hereditary Autosomal Recessive Disorder?
Version of Record online: 12 APR 2011
Copyright © 2011 by the American College of Veterinary Internal Medicine
Journal of Veterinary Internal Medicine
Volume 25, Issue 3, pages 490–496, May/June 2011
How to Cite
Kleiter, M., Högler, S., Kneissl, S., Url, A. and Leschnik, M. (2011), Spongy Degeneration with Cerebellar Ataxia in Malinois Puppies: A Hereditary Autosomal Recessive Disorder?. Journal of Veterinary Internal Medicine, 25: 490–496. doi: 10.1111/j.1939-1676.2011.0720.x
- Issue online: 3 MAY 2011
- Version of Record online: 12 APR 2011
- Submitted November 9, 2010; Revised February 8, 2011; Accepted March 2, 2011.
- Belgian Shepherd;
- Central nervous system;
Background: There is a high incidence of hereditary degenerative diseases of the central nervous system in purebred dogs.
Hypothesis: Cerebellar ataxia in Malinois puppies, caused by degenerative changes that predominate in cerebellar nuclei and the granular cell layer, is a hereditary disorder that is distinct from cerebellar cortical abiotrophies.
Animals: Thirteen Malinois puppies with cerebellar ataxia.
Methods: Retrospective study. Records of Malinois puppies with spongy degeneration of the cerebellar nuclei were analyzed including clinical signs, histopathological changes, and pedigree data.
Results: Signs of cerebellar dysfunction were observed in puppies of both sexes from 5 different litters (1995–2009) of phenotypically normal parents. Clinical signs started before the age of 2 months and resulted in euthanasia of all puppies by the age of 13 weeks. Histopathology disclosed marked bilateral spongy degeneration of the cerebellar nuclei and vacuoles in the granular cell layer and foliate white matter of the cerebellum. In some puppies, discrete vacuoles in gray and white matter were present in other parts of the brain. Furthermore, spheroids and dilated myelin sheaths were observed. Pedigree data and segregation frequency support an autosomal recessive hereditary disorder.
Conclusions and Clinical Importance: Malinois suffer from a hereditary spongiform degeneration that predominates in the cerebellum and causes an early onset of clinical signs with unfavorable prognosis. Future efforts should increase awareness among veterinarians and breeders and aim to identify underlying metabolic mechanisms and the affected genes.