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Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study
  1. K Pugdahl1,
  2. A Fuglsang-Frederiksen1,
  3. M de Carvalho2,
  4. B Johnsen1,
  5. P R W Fawcett3,
  6. A Labarre-Vila4,
  7. R Liguori5,
  8. W A Nix6,
  9. I S Schofield3
  1. 1Department of Clinical Neurophysiology, Aarhus University Hospital, Århus C, Denmark
  2. 2Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal
  3. 3Department of Clinical Neurophysiology, Newcastle General Hospital, Newcastle upon Tyne, UK
  4. 4Département de Neurologie, Centre Hospitalier Universitaire, Grenoble, France
  5. 5Institute of Neurology, University of Bologna, Bologna, Italy
  6. 6Department of Neurology, University Clinics Mainz, Mainz, Germany
  1. Correspondence to:
 A Fuglsang-Frederiksen
 Department of Clinical Neurophysiology, Aarhus University Hospital, Nørrebrogade 44, 8000 Århus C, Denmark; affre{at}as.aaa.dk

Abstract

Background: Amyotrophic lateral sclerosis (ALS) is defined as a disease of the motor neurones, although several studies indicate involvement of the sensory nervous system.

Aim: To evaluate the sensory nerve conduction studies (NCS) in 88 patients with ALS as part of a European multicentre study.

Methods: Seven European clinical neurophysiologists examined consecutive series of ALS patients. The examinations were peer reviewed, and the diagnosis of ALS was confirmed clinically.

Results: 20 (22.7%) patients with ALS had sensory NCS abnormalities in at least one nerve. Of those, 11 (12.5% of all patients) obtained an additional peer review diagnosis of electrophysiological polyneuropathy. There was no difference between the subgroups of patients with normal versus abnormal sensory NCS findings with respect to age, duration and region of onset.

Conclusion: The findings support previous reports of sensory involvement in ALS, and raise the question of whether patients with ALS with sensory nerve abnormalities represent a variant of ALS. ALS associated with generalised sensory system abnormalities may be consistent with degeneration of motor neurones and dorsal root ganglion cells.

  • ALS, amyotrophic lateral sclerosis
  • ESTEEM, European Standardised Telematic Tool to Evaluate Electrodiagnostic Methods
  • GSSA, generalised sensory system abnormalities
  • LMN, lower motor neurone
  • NCS, nerve conduction studies
  • SNAP, sensory nerve action potential
  • SNCV, sensory nerve conduction velocity
  • UMN, upper motor neurone

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Footnotes

  • Competing interests: None declared.

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