2. Academic Validation
  3. Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis

  • Nat Rev Dis Primers. 2017 Oct 5;3:17071. doi: 10.1038/nrdp.2017.71.
Orla Hardiman 1,  Ammar Al-Chalabi 2,  Adriano Chio 3,  Emma M Corr 1,  Giancarlo Logroscino 4,  Wim Robberecht 5,  Pamela J Shaw 6,  Zachary Simmons 7,  Leonard H van den Berg 8
Affiliations

Affiliations

  • 1 Academic Unit of Neurology, Room 5.41 Trinity Biomedical Science Institute, Trinity College Dublin, Pearse Street, Dublin 2, Ireland.
  • 2 Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK.
  • 3 Rita Levi Montalcini Department of Neurosciences, University of Turin, Turin, Italy.
  • 4 Department of Neuroscience, University of Bari, Bari, Italy.
  • 5 KU Leuven-University of Leuven, University Hospitals Leuven, Department of Neurology, Leuven, Belgium.
  • 6 Sheffield Institute for Translational Neuroscience, University of Sheffield, Sheffield, UK.
  • 7 Department of Neurology, Milton S. Hershey Medical Center, Penn State Health, Hershey, Pennsylvania, USA.
  • 8 Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, Utrecht, The Netherlands.
PMID: 28980624 DOI: 10.1038/nrdp.2017.71
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.

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