Nerve Cell Death in Degenerative Diseases of the Central Nervous System: Clinical Aspects

  1. Gregory Bock Organizer and
  2. Maeve O'Connor
  1. Yves Agid and
  2. Jérome Blin

Published Online: 28 SEP 2007

DOI: 10.1002/9780470513422.ch2

Ciba Foundation Symposium 126 - Selective Neuronal Death

Ciba Foundation Symposium 126 - Selective Neuronal Death

How to Cite

Agid, Y. and Blin, J. (2007) Nerve Cell Death in Degenerative Diseases of the Central Nervous System: Clinical Aspects, in Ciba Foundation Symposium 126 - Selective Neuronal Death (eds G. Bock and M. O'Connor), John Wiley & Sons, Ltd., Chichester, UK. doi: 10.1002/9780470513422.ch2

Author Information

  1. Clinique de Neurologie et Neuropsychologie, INSERM U 289, C.H.U. Pitié-Salpětrière, Boulevard de l'Hǒpital, 75634 Paris Cedex 13, France

Publication History

  1. Published Online: 28 SEP 2007

ISBN Information

Print ISBN: 9780471910923

Online ISBN: 9780470513422

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Keywords:

  • nerve cell death;
  • central nervous system;
  • Parkinson's disease;
  • Alzheimer's disease;
  • brain neuronal circuitry

Summary

The origin of degenerative diseases of the central nervous system lies in genetic and acquired disorders. Analysis of the clinical characteristics of diseases affecting specific neuronal systems may help us to understand their pathogenesis. (1) The stereotyped symptomatology characteristic of most degenerative diseases results from neuronal death in specific pathways: pyramidal tract and motor neurons in amyotrophic lateral sclerosis, nigrostriatal dopamine system in Parkinson's disease, posterior and lateral columns of the spinal cord in Friedreich's ataxia, etc. This suggests that these neurons are sensitive to pathological processes that are still unknown. (2) Progression of the disease, whether linear or not, is slow, but it is more rapid than similar effects due to ageing. This indicates either that the environmental cause of degeneration (if it exists) is continuously present or that a vital process has been once and for all disrupted, perhaps at the level of the genome, causing insufficient production of essential proteins, or accumulation of eventually toxic metabolites. (3) Symptoms generally appear during adulthood, i.e. after normal differentiation has taken place, and after a considerable number of neurons have already been damaged. The initiation of neuronal death precedes the appearance of the first symptoms.