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Motor Neuron Disease

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Cell Death and Diseases of the Nervous System

Abstract

Motor neuron disease (MND) refers to the group of degenerative disorders characterized by progressive weakness and atrophy of skeletal muscle due to the selective dysfunction and degeneration of upper and/or lower motor neurons. In adults, the most common of these disorders is amyotrophic lateral sclerosis (ALS, i.e., Lou Gehrig’s disease), which results from the degeneration of large motor neurons in the anterior horn of the spinal cord, brain stem nuclei, and motor cortex. The most common form of MND in children is spinal muscular atrophy (SMA), a distinct form of MND characterized by the dysfunction and loss of anterior horn cells of the spinal cord and lower brainstem nuclei without associated upper motor neuron findings. This chapter will focus on recent advances in understanding the molecular events leading to dysfunction and death of motor neurons in these two disorders.

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Authors
  1. Bruce A. Rabin
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  2. David R. Borchelt
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Editor information

Editors and Affiliations

  1. The Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Vassilis E. Koliatsos MD

  2. Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA, USA

    Rajiv R. Ratan MD, PhD

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© 1999 Springer Science+Business Media New York

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Rabin, B.A., Borchelt, D.R. (1999). Motor Neuron Disease. In: Koliatsos, V.E., Ratan, R.R. (eds) Cell Death and Diseases of the Nervous System. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-4612-1602-5_20

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  • DOI: https://doi.org/10.1007/978-1-4612-1602-5_20

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-4612-7213-7

  • Online ISBN: 978-1-4612-1602-5

  • eBook Packages: Springer Book Archive

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