Skip to main content
NCBI home page
Canadian Journal of Veterinary Research logoLink to Canadian Journal of Veterinary Research
. 1990 Jan;54(1):77–82.

Hereditary canine spinal muscular atrophy: canine motor neuron disease.

L C Cork 1, D L Price 1, J W Griffin 1, G H Sack Jr 1
PMCID: PMC1255609  PMID: 2306675

Abstract

Motor neuron diseases, manifest as weakness and atrophy of skeletal muscles, occur in infancy, childhood, and adult life. Some forms of this disease are inherited. Motor neurons are selectively affected and exhibit cytoskeletal pathology, primarily enlargements of proximal axons by accumulations of transported neurofilaments. A motor neuron disease, hereditary canine spinal muscular atrophy, has been discovered in Brittany spaniels. The disease is inherited as an autosomal dominant characteristic and shows striking clinical and pathological features in common with human motor neuron disease. The availability of this excellent animal model of the human condition has allowed neurobiological investigations of the dynamics of structural and chemical pathologies of vulnerable neurons.

Full text

PDF
77

Images in this article

78Fig. 1.
on p.78

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Andrews J. M., Andrews R. L. The comparative neuropathology of motor neuron diseases. UCLA Forum Med Sci. 1976;(19):181–216. [PubMed] [Google Scholar]
  2. BYERS R. K., BANKER B. Q. Infantile muscular atrophy. Arch Neurol. 1961 Aug;5:140–164. doi: 10.1001/archneur.1961.00450140022003. [DOI] [PubMed] [Google Scholar]
  3. Bradley W. G., Good P., Rasool C. G., Adelman L. S. Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis. Ann Neurol. 1983 Sep;14(3):267–277. doi: 10.1002/ana.410140304. [DOI] [PubMed] [Google Scholar]
  4. Carpenter S. Proximal axonal enlargement in motor neuron disease. Neurology. 1968 Sep;18(9):841–851. doi: 10.1212/wnl.18.9.841. [DOI] [PubMed] [Google Scholar]
  5. Chou S. M., Fakadej A. V. Ultrastructure of chromatolytic motoneurons and anterior spinal roots in a case of Werdnig-Hoffmann disease. J Neuropathol Exp Neurol. 1971 Jul;30(3):368–379. doi: 10.1097/00005072-197107000-00005. [DOI] [PubMed] [Google Scholar]
  6. Clark A. W., Griffin J. W., Price D. L. The axonal pathology in chronic IDPN intoxication. J Neuropathol Exp Neurol. 1980 Jan;39(1):42–55. doi: 10.1097/00005072-198001000-00004. [DOI] [PubMed] [Google Scholar]
  7. Cork L. C., Griffin J. W., Choy C., Padula C. A., Price D. L. Pathology of motor neurons in accelerated hereditary canine spinal muscular atrophy. Lab Invest. 1982 Jan;46(1):89–99. [PubMed] [Google Scholar]
  8. Delisle M. B., Carpenter S. Neurofibrillary axonal swellings and amyotrophic lateral sclerosis. J Neurol Sci. 1984 Feb;63(2):241–250. doi: 10.1016/0022-510x(84)90199-0. [DOI] [PubMed] [Google Scholar]
  9. Griffin J. W., Hoffman P. N., Clark A. W., Carroll P. T., Price D. L. Slow axonal transport of neurofilament proteins: impairment of beta,beta'-iminodipropionitrile administration. Science. 1978 Nov 10;202(4368):633–635. doi: 10.1126/science.81524. [DOI] [PubMed] [Google Scholar]
  10. Hart M. N., Cancilla P. A., Frommes S., Hirano A. Anterior horn cell degeneration and Bunina-type inclusions associated with dementia. Acta Neuropathol. 1977 Jun 15;38(3):225–228. doi: 10.1007/BF00688069. [DOI] [PubMed] [Google Scholar]
  11. Hestnes A., Mellgren S. I. Familial amyotrophic lateral sclerosis. Report of a family with predominant upper limb pareses and late onset. Acta Neurol Scand. 1980 Mar;61(3):192–199. [PubMed] [Google Scholar]
  12. Hoffman P. N., Griffin J. W., Price D. L. Control of axonal caliber by neurofilament transport. J Cell Biol. 1984 Aug;99(2):705–714. doi: 10.1083/jcb.99.2.705. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Johnson J. P., McLean R. H., Cork L. C., Winkelstein J. A. Genetic analysis of an inherited deficiency of the third component of complement in Brittany spaniel dogs. Am J Med Genet. 1986 Nov;25(3):557–562. doi: 10.1002/ajmg.1320250319. [DOI] [PubMed] [Google Scholar]
  14. Juergens S. M., Kurland L. T., Okazaki H., Mulder D. W. ALS in Rochester, Minnesota, 1925-1977. Neurology. 1980 May;30(5):463–470. doi: 10.1212/wnl.30.5.463. [DOI] [PubMed] [Google Scholar]
  15. Lorenz M. D., Cork L. C., Griffin J. W., Adams R. J., Price D. L. Hereditary spinal muscular atrophy in Brittany Spaniels: clinical manifestations. J Am Vet Med Assoc. 1979 Oct 15;175(8):833–839. [PubMed] [Google Scholar]
  16. Sack G. H., Jr, Cork L. C., Morris J. M., Griffin J. W., Price D. L. Autosomal dominant inheritance of hereditary canine spinal muscular atrophy. Ann Neurol. 1984 Apr;15(4):369–373. doi: 10.1002/ana.410150411. [DOI] [PubMed] [Google Scholar]
  17. Tandan R., Bradley W. G. Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol. 1985 Sep;18(3):271–280. doi: 10.1002/ana.410180302. [DOI] [PubMed] [Google Scholar]
  18. Tandan R., Bradley W. G. Amyotrophic lateral sclerosis: Part 2. Etiopathogenesis. Ann Neurol. 1985 Oct;18(4):419–431. doi: 10.1002/ana.410180402. [DOI] [PubMed] [Google Scholar]
  19. Troncoso J. C., Hoffman P. N., Griffin J. W., Hess-Kozlow K. M., Price D. L. Aluminum intoxication: a disorder of neurofilament transport in motor neurons. Brain Res. 1985 Sep 2;342(1):172–175. doi: 10.1016/0006-8993(85)91369-1. [DOI] [PubMed] [Google Scholar]
  20. Whitehouse P. J., Wamsley J. K., Zarbin M. A., Price D. L., Tourtellotte W. W., Kuhar M. J. Amyotrophic lateral sclerosis: alterations in neurotransmitter receptors. Ann Neurol. 1983 Jul;14(1):8–16. doi: 10.1002/ana.410140103. [DOI] [PubMed] [Google Scholar]
  21. Wikström J., Paetau A., Palo J., Sulkava R., Haltia M. Classic amyotrophic lateral sclerosis with dementia. Arch Neurol. 1982 Nov;39(11):681–683. doi: 10.1001/archneur.1982.00510230007002. [DOI] [PubMed] [Google Scholar]
  22. Winkelstein J. A., Cork L. C., Griffin D. E., Griffin J. W., Adams R. J., Price D. L. Genetically determined deficiency of the third component of complement in the dog. Science. 1981 Jun 5;212(4499):1169–1170. doi: 10.1126/science.7233211. [DOI] [PubMed] [Google Scholar]

Articles from Canadian Journal of Veterinary Research are provided here courtesy of Canadian Veterinary Medical Association

RESOURCES