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Shetland Sheepdog leukodystrophy.

S L Wood1, J S Patterson

  • 1Department of Small Animal Clinical Sciences, Michigan State University Veterinary Teaching Hospital, East Lansing, USA. sheridanwood@aol.com

Journal of Veterinary Internal Medicine
|October 13, 2001
PubMed
Summary
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A familial leukodystrophy causing neurological dysfunction was identified in Shetland Sheepdog pups. This inherited condition, resembling Canavan disease, affects the brain and spinal cord

Area of Science:

  • Veterinary Neurology
  • Canine Genetics
  • Neurobiology

Background:

  • Investigated a familial neurological disorder in Shetland Sheepdog litters characterized by seizures and progressive dysfunction.
  • Suspected an inherited basis for the observed neurological syndrome in affected pups.

Observation:

  • Nine pups across three litters exhibited severe, diffuse spongy degeneration of the brain and spinal cord white matter.
  • Computed tomography revealed diffuse hypomyelination in affected individuals.

Findings:

  • Postmortem examinations, including histopathology and electron microscopy, identified a leukodystrophy similar to human Canavan disease.
  • Standard metabolic and biochemical screening tests did not reveal abnormalities in amino acid or organic acid metabolism.
  • The specific etiology for this Shetland Sheepdog leukodystrophy remains undetermined.

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Implications:

  • This study highlights a novel inherited leukodystrophy in the purebred dog population.
  • Further research is needed to elucidate the genetic and molecular basis of this canine neurological disease.
  • Understanding this condition may offer insights into human leukodystrophies like Canavan disease.