ISSN 2398-2969      

Giant axonal neuropathy

icanis
Contributor(s):

Kyle Braund

Laurent Garosi


Introduction

  • Extremely rare.
  • Condition is inherited as an autosomal recessive trait.
  • Recognized in the German Shepherd dog (Alsatian) German Shepherd Dog.
  • Diagnosis: nerve biopsy.
  • Signs: ataxia, paraparesis, regurgitation, laryngeal dysfunction.
  • Treatment: none.
  • Prognosis: poor due to aspiration pneumonia and progressive nature of deficits.

Pathogenesis

Predisposing factors

General
  • Presently unknown.

Specific

  • Giant axonal neuropathy developed after mating of consanguineous phenotypically normal parents. The mating was repeated with similar consequences. These results suggested that inheritance pattern for canine giant axonal neuroapthy is autosomal recessive.

Pathophysiology

  • Not presently well understood.
  • Lesions have been characterized in the CNS and PNS.
  • Neurogenic atrophy was evident in muscles below the stifle joint.
  • The massive accumulation of neurofilaments in distal portions of nerves as well as in long motor and sensory tracts of the spinal cord/brain stem suggests a defect in axoplasmic transport which may result from an energy failure.
  • The neurofilamentous accumulations result in multifocal axonal swellings and eventual axonal degeneration.
  • The distribution of lesions suggests this condition represents a central-peripheral distal axonopathy.

Timecourse

  • Progressive deterioration so that megaesophagus Megaesophagus , muscle atrophy, tetraparesis/tetraplegia typically develop by 18-24 months of age.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Coates J R, O'Brien D P (2004) Inherited peripheral neuropathies in dogs and cats. Vet Clin Small Anim 34, 1361-1401 PubMed.
  • Braund K G (1996) Degenerative causes of neuropathies in dogs and cats. Vet Med 91 (8), 722-739 VetMedResource.
  • King R H M et al (1993) Axonal neurofilamentous accumulations - a comparison between human and canine axonal neuropathy and 2,5-HD neuropathy. Neuropathol Appl Neurobiol 19 (3), 224-232 PubMed.
  • Duncan I D et al (1981) Inherited giant axonal neuropathy. Muscle Nerve 4, 223-227 PubMed.
  • Duncan I D & Griffiths I R (1981) Canine giant axonal neuropathy; some aspects of its clinical, pathological and comparative features. JSAP 22 (8), 491-501 PubMed.
  • Griffiths I R et al (1980) Further studies of the central nervous system in canine giant axonal neuropathy. Neuropathol Appl Neurobiol (6), 421-432 PubMed.
  • Duncan I D & Griffiths I R (1977) Canine giant axonal neuropathy. Vet Rec 101 (22), 438-441 PubMed.

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