Skip to main content
NCBI home page
Journal of Medical Genetics logoLink to Journal of Medical Genetics
. 1974 Mar;11(1):76–79. doi: 10.1136/jmg.11.1.76

A Neurogenic Component in Muscular Dystrophy

Alan E H Emery 1, Christine Gosden 1
PMCID: PMC1013091  PMID: 4600009

Abstract

Evidence for a neurogenic component in mouse and human muscular dystrophy is briefly reviewed. Such evidence comes from certain clinical observations, electrophysiological studies, muscle pathology, nervous system pathology, transplantation experiments in animals, and tissue culture studies. The evidence is at present rather conflicting though the results of recent tissue culture experiments are more convincing. If there is a neurogenic component in dystrophy then the basic defect may have to be sought in the central nervous system rather than in the muscle itself. It is argued, however, that a neurogenic component in dystrophy cannot be simply a defect in the anterior horn cells of the spinal cord since the clinical features and the laboratory and pathological findings are quite different from those in spinal muscular atrophy.

Full text

PDF
76

Selected References

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

  1. Askanas V., Shafiq S. A., Milhorat A. T. Histochemistry of cultured aneural chick muscle. Morphological maturation without differentiation of fiber types. Exp Neurol. 1972 Oct;37(1):218–230. doi: 10.1016/0014-4886(72)90238-5. [DOI] [PubMed] [Google Scholar]
  2. Bateson R. G., Warren J., Hindle D. Growth pattern in vitro of normal and diseased adult human skeletal muscle. J Neurol Sci. 1972 Feb;15(2):183–191. doi: 10.1016/0022-510x(72)90006-8. [DOI] [PubMed] [Google Scholar]
  3. Bishop A., Gallup B., Skeate Y., Dubowitz V. Morphological studies on normal and diseased human muscle in culture. J Neurol Sci. 1971 Jul;13(3):333–350. doi: 10.1016/0022-510x(71)90037-2. [DOI] [PubMed] [Google Scholar]
  4. Bradley W. G., Jaros E. Axoplasmic flow in axonal neuropathies. II. Axoplasmic flow in mice with motor neuron disease and muscular dystrophy. Brain. 1973 Jun;96(2):247–258. doi: 10.1093/brain/96.2.247. [DOI] [PubMed] [Google Scholar]
  5. Bradley W. G., Jenkison M. Abnormalities of peripheral nerves in murine muscular dystrophy. J Neurol Sci. 1973 Feb;18(2):227–247. doi: 10.1016/0022-510x(73)90009-9. [DOI] [PubMed] [Google Scholar]
  6. Dastur D. K., Razzak Z. A. Possible neurogenic factor in muscular dystrophy: its similarity to denervation atrophy. J Neurol Neurosurg Psychiatry. 1973 Jun;36(3):399–410. doi: 10.1136/jnnp.36.3.399. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Emery A. E. The nosology of the spinal muscular atrophies. J Med Genet. 1971 Dec;8(4):481–495. doi: 10.1136/jmg.8.4.481. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Gallup B., Dubowitz V. Letter: Failure of "dystrophic" neurones to support functional regeneration of normal or dystrophic muscle in culture. Nature. 1973 Jun 1;243(5405):287–289. doi: 10.1038/243287a0. [DOI] [PubMed] [Google Scholar]
  9. Gallup B., Strugalska-Cynowska H., Dubowitz V. Histochemical studies on normal and diseased human and chick muscle in tissue culture. J Neurol Sci. 1972 Oct;17(2):109–125. doi: 10.1016/0022-510x(72)90134-7. [DOI] [PubMed] [Google Scholar]
  10. Harris J. B., Wallace C., Wing J. Myelinated nerve fibre counts in the nerves of normal and dystrophic mouse muscle. J Neurol Sci. 1972 Mar;15(3):245–249. doi: 10.1016/0022-510x(72)90067-6. [DOI] [PubMed] [Google Scholar]
  11. Harris J., Wilson P. Denervation in murine dystrophy. Nature. 1971 Jan 1;229(5279):61–62. doi: 10.1038/229061a0. [DOI] [PubMed] [Google Scholar]
  12. Hironaka T., Miyata Y. Muscle transplantation in the aetiological elucidation of murine muscular dystrophy. Nat New Biol. 1973 Aug 15;244(137):221–223. doi: 10.1038/newbio244221a0. [DOI] [PubMed] [Google Scholar]
  13. Hofmann W. W., Thesleff S. Studies on the trophic influence of nerve on skeletal muscle. Eur J Pharmacol. 1972 Dec;20(3):256–260. doi: 10.1016/0014-2999(72)90182-3. [DOI] [PubMed] [Google Scholar]
  14. Jedrzejczyk J., Wieckowski J., Rymaszewska T., Barnard E. A. Dystrophic chicken muscle: altered synaptic acetylcholinesterase. Science. 1973 Apr 27;180(4084):406–408. doi: 10.1126/science.180.4084.406. [DOI] [PubMed] [Google Scholar]
  15. Laird J. L., Timmer R. F. Transplantation of skeletal muscle into a host with muscular dystrophy. Tex Rep Biol Med. 1966 Summer;24(2):169–179. [PubMed] [Google Scholar]
  16. Lin J., Hartmann H. A. Nuclear and cytoplasmic RNA in normal and dystrophic neurons. Brain Res. 1971 Dec 24;35(2):397–407. doi: 10.1016/0006-8993(71)90483-5. [DOI] [PubMed] [Google Scholar]
  17. McComas A. J., Sica R. E., Campbell M. J. "Sick" motoneurones. A unifying concept of muscle disease. Lancet. 1971 Feb 13;1(7694):321–326. doi: 10.1016/s0140-6736(71)91045-2. [DOI] [PubMed] [Google Scholar]
  18. Papapetropoulos T. A., Bradley W. G. Spinal motor neurones in murine muscular dystrophy and spinal muscular atrophy. A quantitative histological study. J Neurol Neurosurg Psychiatry. 1972 Feb;35(1):60–65. doi: 10.1136/jnnp.35.1.60. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Ragab A. H. Motor-end-plate changes in mouse muscular dystrophy. Lancet. 1971 Oct 9;2(7728):815–816. doi: 10.1016/s0140-6736(71)92764-4. [DOI] [PubMed] [Google Scholar]
  20. Rolston J. L. Muscle transplantation into mice with muscular dystrophy. Arch Neurol. 1972 Mar;26(3):258–264. doi: 10.1001/archneur.1972.00490090084007. [DOI] [PubMed] [Google Scholar]
  21. Rosman N. P., Kakulas B. A. Mental deficiency associated with muscular dystrophy. A neuropathological study. Brain. 1966 Dec;89(4):769–788. doi: 10.1093/brain/89.4.769. [DOI] [PubMed] [Google Scholar]
  22. Salafsky B. Functional studies of reeenerated muscles from normal and dystrophic mice. Nature. 1971 Jan 22;229(5282):270–272. doi: 10.1038/229270a0. [DOI] [PubMed] [Google Scholar]
  23. Swash M. The morphology and innervation of the muscle spindle in dystrophia myotonica. Brain. 1972;95(2):357–368. doi: 10.1093/brain/95.2.357. [DOI] [PubMed] [Google Scholar]
  24. Zellweger H., Hanson J. W. Psychometric studies in muscular dystrophy type 3a (Duchenne). Dev Med Child Neurol. 1967 Oct;9(5):576–581. doi: 10.1111/j.1469-8749.1967.tb02327.x. [DOI] [PubMed] [Google Scholar]
  25. Zellweger H., Niedermeyer E. Central nervous system manifestations in childhood muscular dystrophy (CMD). I. Psychometric and electroencephalographic findings. Ann Paediatr. 1965;205(1):25–42. [PubMed] [Google Scholar]

Articles from Journal of Medical Genetics are provided here courtesy of BMJ Publishing Group

RESOURCES