Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 78; no. 7; pp. 746 - 749
• Main Authors: Pugdahl, K, Fuglsang-Frederiksen, A, de Carvalho, M, Johnsen, B, Fawcett, P R W, Labarre-Vila, A, Liguori, R, Nix, W A, Schofield, I S
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group Ltd 01.07.2007; BMJ; BMJ Publishing Group LTD; BMJ Publishing Group; BMJ Group
• Subjects: Adult; Aged; Aged, 80 and over; ALS; Amyotrophic Lateral Sclerosis; Amyotrophic Lateral Sclerosis - physiopathology; Biological and medical sciences; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Diabetes; Electrophysiology; ESTEEM; Europe; Europe - epidemiology; European Standardised Telematic Tool to Evaluate Electrodiagnostic Methods; Female; generalised sensory system abnormalities; GSSA; Humans; Life Sciences; LMN; lower motor neurone; Male; Medical sciences; Middle Aged; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; NCS; nerve conduction studies; Nervous system; Neural Conduction; Neurology; Neurons and Cognition; Neurons, Afferent; Neurons, Afferent - physiology; Patients; Peer review; Physicians; Sensation Disorders; Sensation Disorders - epidemiology; sensory nerve action potential; sensory nerve conduction velocity; Short Report; SNAP; SNCV; UMN; upper motor neurone; Europe; Amyotrophic lateral sclerosis; Nervous system diseases; Degenerative disease; Spinal cord disease; Central nervous system disease
• ISSN: 0022-3050; 1468-330X
• DOI: 10.1136/jnnp.2006.098533

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.