Amyotrophic lateral sclerosis

Orla Hardiman, Ammar Al-Chalabi, Adriano Chio, Emma M. Corr, Giancarlo Logroscino, Wim Robberecht, Pamela J. Shaw, Zachary Simmons, Leonard H. Van Den Berg

Research output: Contribution to journalReview articlepeer-review

311 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease, is characterized by the degeneration of both upper and lower motor neurons, which leads to muscle weakness and eventual paralysis. Until recently, ALS was classified primarily within the neuromuscular domain, although new imaging and neuropathological data have indicated the involvement of the non-motor neuraxis in disease pathology. In most patients, the mechanisms underlying the development of ALS are poorly understood, although a subset of patients have familial disease and harbour mutations in genes that have various roles in neuronal function. Two possible disease-modifying therapies that can slow disease progression are available for ALS, but patient management is largely mediated by symptomatic therapies, such as the use of muscle relaxants for spasticity and speech therapy for dysarthria.

Original languageEnglish (US)
Article number17071
JournalNature Reviews Disease Primers
Volume3
DOIs
StatePublished - Oct 5 2017

All Science Journal Classification (ASJC) codes

  • Medicine(all)

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