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Review
Split-hand and split-limb phenomena in amyotrophic lateral sclerosis: pathophysiology, electrophysiology and clinical manifestations
  1. Philippe Corcia1,
  2. Peter Bede2,
  3. Pierre-François Pradat3,4,
  4. Philippe Couratier5,
  5. Steve Vucic6,7,
  6. Mamede de Carvalho8,9
  1. 1Centre Constitutif de Référence SLA, CHU Bretonneau, Tours, France
  2. 2Boulevard Tonnellé, Tours, France
  3. 3Neurology, Hopital Pitie-Salpetriere, Paris, France
  4. 4LIB, Université Pierre et Marie Curie Faculté de Médecine, Paris, Île-de-France, France
  5. 5Centre Constitutif de Référence SLA, CHU de Limoges, Limoges, France
  6. 6Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney, New South Wales, Australia
  7. 7Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia
  8. 8Instituto de Fisiologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisbon, Lisbon, Portugal
  9. 9Department of Neurosciences and Mental Health, Hospital de Santa Maria, Lisboa, Portugal
  1. Correspondence to Professor Philippe Corcia, ALS Center, Université de Tours Faculté de Médecine, Tours, Centre-Val de Loire, France; corcia{at}med.univ-tours.fr

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder affecting the upper and lower motor neurons. A key clinical feature of ALS is the absence of accurate, early-stage diagnostic indicators. ‘Split-hand syndrome’ was first described in ALS at the end of the last century and a considerable body of literature suggests that the split-hand phenomenon may be an important clinical feature of ALS. Considering the published investigations, it is conceivable that the ‘split-hand syndrome’ results from the associated upper and lower motor neuron degeneration, whose interaction remains to be fully clarified. Additionally, other split syndromes have been described in ALS involving upper or lower limbs, with a nuanced description of clinical and neurophysiological manifestations that may further aid ALS diagnosis. In this review, we endeavour to systematically present the spectrum of the ‘split syndromes’ in ALS from a clinical and neurophysiology perspective and discuss their diagnostic and pathogenic utility.

  • motor neuron disease
  • neurophysiol
  • clinical
  • clinical neurology

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Footnotes

  • Contributors All authors contributed equally to the inception and drafting of this review paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.