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Table of Contents
Vol. 2, No. 3-4, 2005
Issue release date: January 2006
Section title: Toxicity Pathways in ALS
Neurodegenerative Dis 2005;2:147–159
(DOI:10.1159/000089620)

Excitotoxicity and Amyotrophic Lateral Sclerosis

van Cutsem P. · Dewil M. · Robberecht W. · van den Bosch L.
Neurobiology, Campus Gasthuisberg, Leuven, Belgium

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Article / Publication Details

First-Page Preview
Abstract of Toxicity Pathways in ALS

Published online: January 06, 2006
Issue release date: January 2006

Number of Print Pages: 13
Number of Figures: 2
Number of Tables: 0

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD

Abstract

Since its description by Charcot more than 130 years ago, the pathogenesis of selective motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remains unsolved. Over the years, many pathogenic mechanisms have been proposed. Amongst others these include: oxidative stress, excitotoxicity, aggregate formation, inflammation, growth factor deficiency and neurofilament disorganization. This multitude of contributing factors indicates that ALS is a complex disease and also suggests that ALS is a multifactorial disorder. Excitotoxicity is not the newest and most spectacular hypothesis in the ALS field, but it is undoubtedly one of the most robust pathogenic mechanisms supported by an impressive amount of evidence. Moreover, the therapeutic efficacy of riluzole, the only drug proven to slow disease progression in ALS, is most likely related to its anti-excitotoxic properties. In this review, we will give an overview of the arguments in favor of the involvement of excitotoxicity in ALS and of the possible mechanisms leading to motor neuron death. We will also summarize the intrinsic properties of motor neurons that render these cells particularly vulnerable to excitotoxicity and could explain the selective vulnerability of motor neurons in ALS. All this information could help to develop new and better therapeutic strategies that could protect motor neurons from excitotoxicity.

© 2005 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Toxicity Pathways in ALS

Published online: January 06, 2006
Issue release date: January 2006

Number of Print Pages: 13
Number of Figures: 2
Number of Tables: 0

ISSN: 1660-2854 (Print)
eISSN: 1660-2862 (Online)

For additional information: http://www.karger.com/NDD


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