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  • Updated 06.28.2019
  • Released 06.21.1996
  • Expires For CME 06.28.2022

Amyotrophic lateral sclerosis

Introduction

Overview

Amyotrophic lateral sclerosis is a devastating neurodegenerative disease characterized by progressive muscle weakness without notable sensory loss. There are two U.S. Food and Drug Administration-approved medications. Both have demonstrated modest effects on slowing disease progression: riluzole, approved in 1995, and edaravone, approved in 2017. In this article, the author reviews clinical manifestations, risk factors, symptomatic management, and clinical trials and provides updates on recent genetic discoveries.

Historical note and terminology

Aran believed this syndrome was a muscular disease and was the first to use the term “progressive muscular atrophy” (12). Cruveilhier first noticed the atrophy of the anterior spinal roots and thought progressive muscular atrophy was a myelopathic disorder (Cruveilhier 1853). Charcot and Joffroy proposed the term “amyotrophic lateral sclerosis” when they noticed the involvement of the corticospinal tract (32). Brain used the term “motor neuron disease” to emphasize the connections between progressive muscular atrophy, amyotrophic lateral sclerosis, and progressive bulbar palsy (24). The term “motor neuron disease” also highlights the variety of involvement of upper and lower motor neurons. Rowland suggested using the plural form, “motor neuron diseases,” to describe all of the diseases of the anterior horn cells and the motor system, including spinal muscular atrophies (157). Spinal muscular atrophies are clinically and pathologically distinct from amyotrophic lateral sclerosis.

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