2. Academic Validation
  3. Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis

  • Lancet. 2022 Oct 15;400(10360):1363-1380. doi: 10.1016/S0140-6736(22)01272-7.
Eva L Feldman 1,  Stephen A Goutman 2,  Susanne Petri 3,  Letizia Mazzini 4,  Masha G Savelieff 2,  Pamela J Shaw 5,  Gen Sobue 6
Affiliations

Affiliations

  • 1 Department of Neurology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA. Electronic address: [email protected].
  • 2 Department of Neurology, Michigan Medicine, University of Michigan, Ann Arbor, MI, USA.
  • 3 Department of Neurology, Hannover Medical School, Hannover, Germany.
  • 4 ALS Centre, Azienda Ospedaliero-Universitaria Maggiore della Carità, Novara, Italy; Department of Neurology, University of Piemonte Orientale, Novara, Italy.
  • 5 Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, Sheffield, UK.
  • 6 Department of Neurology, Aichi Medical University, Nagakute, Aichi, Japan.
PMID: 36116464 DOI: 10.1016/S0140-6736(22)01272-7
Abstract

Amyotrophic lateral sclerosis is a fatal CNS neurodegenerative disease. Despite intensive research, current management of amyotrophic lateral sclerosis remains suboptimal from diagnosis to prognosis. Recognition of the phenotypic heterogeneity of amyotrophic lateral sclerosis, global CNS dysfunction, genetic architecture, and development of novel diagnostic criteria is clarifying the spectrum of clinical presentation and facilitating diagnosis. Insights into the pathophysiology of amyotrophic lateral sclerosis, identification of disease biomarkers and modifiable risks, along with new predictive models, scales, and scoring systems, and a clinical trial pipeline of mechanism-based therapies, are changing the prognostic landscape. Although most recent advances have yet to translate into patient benefit, the idea of amyotrophic lateral sclerosis as a complex syndrome is already having tangible effects in the clinic. This Seminar will outline these insights and discuss the status of the management of amyotrophic lateral sclerosis for the general neurologist, along with future prospects that could improve care and outcomes for patients with amyotrophic lateral sclerosis.

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