Current and potential therapeutics in motor neuron diseases

doi:10.1017/CBO9780511544873.053

52 - Current and potential therapeutics in motor neuron diseases

from Part IX - Motor neuron diseases

Published online by Cambridge University Press: 04 August 2010

Anthony E. Lang and

Clare Wood-Allum and

M. Flint Beal: Affiliation:
Cornell University, New York
Anthony E. Lang: Affiliation:
University of Toronto
Albert C. Ludolph: Affiliation:
Universität Ulm, Germany
Clare Wood-Allum: Affiliation:
Academic Neurology Unit, Sheffield University Medical School, UK
Pamela J. Shaw: Affiliation:
Academic Neurology Unit, Sheffield University Medical School, UK

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Summary

Introduction

This chapter will survey the research underpinning current therapeutic candidates for amyotrophic lateral sclerosis (ALS), hereditary spastic paraplegia (HSP), Kennedy's disease (SBMA) and spinal muscular atrophy (SMA). It will also review the evidence base and rationale for the use of riluzole in ALS, the only disease-modifying agent currently licensed for use in any neurodegenerative disease of the motor neuron. In the absence of effective therapies to slow disease progression, the focus of management must be on symptomatic therapies aimed at improving quality of life. Guidelines for the symptomatic management of ALS have been generated by the American Academy of Neurology (Miller et al., 1999b) and a number of systematic reviews of the symptomatic management of ALS are also available from the Cochrane library – symptomatic therapies will not be further discussed here (Annane et al., 2000; Langmore et al., 2003). In recent years, improvements in materials science, bioinformatics and the development of exciting new techniques in molecular biology have brought about a revolution in the way that therapeutic candidates are selected and tested. The likely impact of these new techniques on drug development for disorders of motor neurons will also be discussed.

Disease modifying therapies for amyotrophic lateral sclerosis

Aims of therapy

Motor neurons are post-mitotic cells, which make numerous, complex synaptic connections. They are found in the motor cortex, the brainstem and along the entire length of the spinal cord.

Type: Chapter
Information: Neurodegenerative Diseases
Neurobiology, Pathogenesis and Therapeutics
, pp. 772 - 793

DOI: https://doi.org/10.1017/CBO9780511544873.053 [Opens in a new window]

Publisher: Cambridge University Press

Print publication year: 2005

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