Skip to main content

Degenerative Disorders

  • Chapter
Textbook of Pediatric Neurology

Part of the book series: Topics in Pediatrics ((TIPE))

Abstract

The hallmark of degenerative disorders of the nervous system is the progressive loss of previously acquired abilities. In young children, deceleration in the rate of development is often the first sign of degeneration. The patient falls progressively behind other children, and subsequently there is actual loss of previously acquired achievements. This is quantitated as a declining developmental quotient. By definition, this deterioration is not due to an exogenous agent or event, or to secondary involvement of the nervous system by a generalized systemic disease. Most of the degenerative disorders are hereditary, although the biochemical basis of many of these conditions is not fully understood.

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Farrell, D. F., and Ochs, U., 1981, Gm, gangliosidosis: Phenotypic variation in a single family, Ann. Neurol. 9: 225–231.

    Article  PubMed  CAS  Google Scholar 

  2. Farrell, D. F., and MacMartin, M. P., 1981, GM, gangliosidosis: Enzymatic variation in a single family, Ann. Neurol. 9: 232–286.

    Article  PubMed  CAS  Google Scholar 

  3. Miloy, C. E., Gilbert, E. F., France, T. D., O’Brien, J. F., and Chun, R. W. M., 1978, Clinical and extrancural histologic diagnosis of neuronal ceroid-lipofuscinosis, Neurology 28: 1008–1012.

    Google Scholar 

  4. Martin, J. J., Centerick, C., and Libert, J., 1980, Skin and conjunctival nerve biopsies in adrenoleukodystrophy and its variants, Ann. Neurol. 8: 291–295.

    Article  PubMed  CAS  Google Scholar 

  5. Moser, H. W., Moser, A. E., Trojak, J. E., and Supplee, S. W., 1983, Identification of female carriers of adrenoleukodystrophy, J. Pediatr. 103: 54–59.

    Article  PubMed  CAS  Google Scholar 

  6. Moser, H. W., Moser, A. E., Singh, I., and O’Neill, B. P., 1984, Adrenoleukodystrophy: Survey of 303 cases: Biochemistry, diagnosis, and therapy, Ann. Neurol. 16: 628–641.

    Article  PubMed  CAS  Google Scholar 

  7. von Specht, B. U., Geiger, B., Arnon, R., Passwell, J., Keren, G., Goldman, B., and Padeh, B., 1979, Enzyme replacement in Tay—Sachs disease, Neurology 28: 848–854.

    Google Scholar 

Additional Reading

  • Dyken, P., and Krawiecki, N., 1983, Neurodegencrative diseases of infancy and childhood, Ann. Neurol. 13: 351–364.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 1987 Plenum Publishing Corporation

About this chapter

Cite this chapter

Golden, G.S. (1987). Degenerative Disorders. In: Textbook of Pediatric Neurology. Topics in Pediatrics. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7029-1_18

Download citation

  • DOI: https://doi.org/10.1007/978-1-4684-7029-1_18

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-7031-4

  • Online ISBN: 978-1-4684-7029-1

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics