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Peer-reviewed veterinary case report

Hereditary cerebellar ataxia in Malinois puppies causing unsteady walk

By Kleiter, M et al.·Published in Journal of veterinary internal medicine·2011·Department for Companion Animals and Horses·View original on PubMed

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Original publication title: Spongy degeneration with cerebellar ataxia in Malinois puppies: a hereditary autosomal recessive disorder?

Species:
dog

Plain-English summary

A group of Malinois puppies developed serious coordination problems, known as cerebellar ataxia, before they were 2 months old. Sadly, all 13 puppies from five different litters had to be euthanized by 13 weeks due to the severity of their symptoms. Tests showed that the puppies had significant damage in the cerebellum, which is the part of the brain that controls movement. This condition is believed to be a hereditary disorder passed down from their parents, who appeared normal. Unfortunately, there is currently no treatment available, and the prognosis is poor for affected puppies.

People also search for: Malinois puppy coordination problems · cerebellar ataxia in puppies · hereditary brain disorders in dogs

Abstract

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.

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Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/21488963/