Polyneuropathy in Young Siberian Huskies Caused by Degenerative and Inflammatory Diseases

Vet Pathol. 2020 Sep;57(5):666-674. doi: 10.1177/0300985820934112. Epub 2020 Jun 24.

Abstract

Polyneuropathy is defined as the simultaneous dysfunction of several peripheral nerves. In dogs, a number of breeds are predisposed to a variety of immune-mediated and/or degenerative inherited forms of polyneuropathy, with laryngeal paralysis and/or megaesophagus as important clinical features of many of these conditions. This case series describes degenerative and inflammatory polyneuropathies in 7 young Siberian huskies that were categorized based on clinicopathological characteristics as follows: (1) slowly progressive laryngeal paralysis and megaesophagus caused by primary axonal degeneration with large fiber loss (n = 2); (2) slowly progressive polyneuropathy without megaesophagus or laryngeal paralysis caused by primary axonal degeneration with large fiber loss (n = 2); (3) acute inflammatory demyelinating neuropathy causing sensory, motor and autonomic nerve deficits (n = 2); and (4) ganglioradiculitis (sensory neuronopathy; n = 1). Based on the predominantly young age at onset, slow progression, relatedness of affected dogs, and clinical and pathological similarities with inherited neuropathies reported in other dog breeds, a hereditary basis for the degenerative polyneuropathies in Siberian huskies is suspected. However, 5 different mutations in 3 genes known to cause polyneuropathy in other dog breeds (NDRG1, ARHGEF10, or RAB3GAP1) were not detected in the affected Siberian huskies suggesting that more genetic variants remain to be identified. This study highlights the varied underlying lesions of polyneuropathies in young Siberian huskies.

Keywords: Siberian husky; axonal degeneration; demyelination; inflammation; inherited; laryngeal paralysis; megaesophagus; peripheral nervous system diseases.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Demyelinating Diseases
  • Dog Diseases / genetics*
  • Dog Diseases / pathology
  • Dogs
  • Esophageal Achalasia / pathology
  • Esophageal Achalasia / veterinary*
  • Female
  • Genetic Predisposition to Disease
  • Genetic Variation
  • Inflammation / pathology
  • Inflammation / veterinary*
  • Male
  • Mutation
  • Peripheral Nerves / pathology
  • Polyneuropathies / genetics
  • Polyneuropathies / pathology
  • Polyneuropathies / veterinary*
  • Vocal Cord Paralysis / pathology
  • Vocal Cord Paralysis / veterinary*