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Review
The expanding syndrome of amyotrophic lateral sclerosis: a clinical and molecular odyssey
  1. Martin R Turner1,
  2. Michael Swash2,3
  1. 1Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
  2. 2Queen Mary University of London, London, UK
  3. 3University of Lisbon, Lisbon, Portugal
  1. Correspondence to Dr Martin Turner, Nuffield Department of Clinical Neurosciences, West Wing Level 3, John Radcliffe Hospital, Oxford OX3 9DU, USA; martin.turner{at}ndcn.ox.ac.uk

Abstract

Recent advances in understanding amyotrophic lateral sclerosis (ALS) have delivered new questions. Disappointingly, the initial enthusiasm for transgenic mouse models of the disease has not been followed by rapid advances in therapy or prevention. Monogenic models may have inadvertently masked the true complexity of the human disease. ALS has evolved into a multisystem disorder, involving a final common pathway accessible via multiple upstream aetiological tributaries. Nonetheless, there is a common clinical core to ALS, as clear today as it was to Charcot and others. We stress the continuing relevance of clinical observations amid the increasing molecular complexity of ALS.

  • MOTOR NEURON DISEASE
  • CLINICAL NEUROLOGY
  • GENETICS
  • MOLECULAR BIOLOGY
  • PRION

This is an Open Access article distributed in accordance with the terms of the Creative Commons Attribution (CC BY 4.0) license, which permits others to distribute, remix, adapt and build upon this work, for commercial use, provided the original work is properly cited. See: http://creativecommons.org/licenses/by/4.0/

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