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Inherited canine giant axonal neuropathy
Muscle & Nerve
|May 1, 1981
Summary
Giant axonal neuropathy (GAN) in dogs is rare, but this study identified 3 new cases in a second litter. Affected dogs showed progressive hind leg weakness and megaesophagus, confirming GAN with nerve biopsy findings.
Area of Science:
- Veterinary Neurology
- Canine Genetics
- Neurodegenerative Diseases
Background:
- Canine giant axonal neuropathy (GAN) is a rare neurodegenerative disorder.
- Only one previous case of canine GAN has been documented.
- This study investigates a new familial occurrence of GAN.
Purpose of the Study:
- To describe the clinical and pathological findings of giant axonal neuropathy (GAN) in a new cohort of dogs.
- To further characterize the presentation and progression of GAN in canines.
- To investigate the genetic basis of GAN in affected litters.
Main Methods:
- Clinical and electrophysiological examinations of 11 dogs from a second litter.
- Longitudinal monitoring for neurological and gastrointestinal signs.
- Nerve biopsy (tibial fascicular nerve) for histopathological analysis.
Main Results:
- Three dogs developed progressive hind limb ataxia and lower motor neuron signs starting at 14-16 months.
- Megaesophagus and regurgitation were observed in affected dogs.
- Electrophysiology showed reduced evoked muscle action potential amplitude; nerve biopsies revealed swollen neurofilaments in unmyelinated and myelinated fibers.
Conclusions:
- This report expands the known cases of canine giant axonal neuropathy (GAN).
- GAN in dogs presents with progressive motor deficits and megaesophagus.
- Histopathological findings confirm axonal swelling and neurofilament accumulation characteristic of GAN.