Skip to main content
Log in

How do neurons degenerate in transmissible spongiform encephalopathies?

  • Part X Neuropathology: Clinical and Experimental Aspects
  • Published:
Molecular and chemical neuropathology

Abstract

Neuroaxonal dystrophy is a feature of neuronal degeneration encountered in all subacute spongiform virus encephalopathies including scrapie and Creutzfeldt-Jakob disease (CJD). By immunohistochemical techniques, the accumulation of 200 kDa neurofilament protein was demonstrated in affected neurites in human CJD. These neurites exhibited the ultrastructural features of dystrophic neurites encountered in other neurodegenerative disorders, particularly Alzheimer's disease. These findings support the hypothesis that impairment of slow axoplasmic transport is a common pathogenetic mechanism for CJD and many other neurodegenerative conditions.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Clark A. W., Parhard I. M., Griffin J. W., and Price D. L. (1984) Neurofilamentous axonal swellings as a normal finding in the spinal anterior horn of man and other primates.J. Neuropathol. Exp. Neurol. 43, 253–262.

    Article  CAS  Google Scholar 

  • Gajdusek D. C. (1984) Interference with axonal transport of neurofilaments as the common etiology and pathogenesis of neurofibrillary tangles, amyotrophic lateral sclerosis, parkinsonism-dementia, and many other degenerations of the cns: a series of hypothesis, inAmyotrophic Lateral Sclerosis in Asia and Oceania.Proceedings of the Sixth Asian and Oceanian Congress of Neurology. Amyotrophic Lateral Sclerosis Workshop, Taipei, Taiwan, Republic of China, Nov. 14, 1983 (Chen K. M., Yase Y., eds.), Shyan-Fu Chou, National Taiwan University, pp. 423–436.

    Google Scholar 

  • Gajdusek D. C. (1985a) Hypothesis: interference with axonal transport of neurofilaments as a common pathogenetic mechanism in certain diseases of the central nervous system.N. Engl. J. Med. 209, 714–719.

    Article  Google Scholar 

  • Gajdusek D. C. (1985b) Interference with axonal transport of neurofilaments as a mechanism of pathogenesis underlying Alzheimer's disease and many other degenerations of the CNS, inNormal Aging, Alzheimer's Disease and Senile Dementia. Aspects of Etiology, Pathogenesis, Diagnosis and Treatment (Gottfries C. G., ed.), Edititions de l'Universite Bruxelles, Bruxelles, pp. 51–67.

    Google Scholar 

  • Gajdusek D. C. (1987) A newly recognized mechanism of pathogenesis in Alzheimer's disease, amyotrophic lateral sclerosis, and other degenerative neurological diseases: the beta-fibrilloses of brain, inNutrition, Health and Peace. Proc. Int. Symp. Honor of Linus Pauling (Jariwalla R. J. and Schwoebel S. L., eds.), Linus Pauling Inst., Palo Alto, pp. 21–55.

    Google Scholar 

  • Jeffrey M., Fraser H., Halliday W. G., Fowler N., Goodsir C. M., and Brown D. A. (1995) Early unsuspected neuron and axon terminals loss in scrapie-infected mice revealed by morphometry and immunocytochemistry.Neuropathol. Appl. Neurobiol. 21, 41–49.

    Article  CAS  Google Scholar 

  • Liberski P. P. and Budka H. (1992) An overview of neuropathology of the slow unconventional virus infectios, inLight and Electron Microscopic Neuropathology of Slow Virus Infections, (Liberski P. P., ed.), CRC, Boca Raton, FL, pp. 111–153.

    Google Scholar 

  • Liberski P. P., Budka H., Yanagihara R., and Gajdusek D. C. (1995) Neuroaxonal dystrophy in experimental Creutzfeldt-Jakob disease: electron microscopical and immunohistochemical demonstration of neurofilament accumulations within affected neurites.J. Comp. Pathol. 112, 243–255.

    Article  CAS  Google Scholar 

  • Schlapfer W. W. (1987) Neurofilaments: structure, metabolism and implications in disease.J. Neuropathol. Exp. Neurol. 46, 117–129.

    Article  Google Scholar 

  • Smith M. C. (1955) Argyrophilic bodies in the human spinal cord.J. Neurol. Neurosurg. Psychiatr. 18, 13–16.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Liberski, P.P. How do neurons degenerate in transmissible spongiform encephalopathies?. Molecular and Chemical Neuropathology 28, 245–249 (1996). https://doi.org/10.1007/BF02815229

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02815229

Index Entries

Navigation