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Diagnostic criteria for amyotrophic lateral sclerosis: A multicentre study of inter-rater variation and sensitivity

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  • B. Johnsen
  • K. Pugdahl
  • A. Fuglsang-Frederiksen
  • K. Kollewe, Hannover Medical School
  • ,
  • L. Paracka, Hannover Medical School
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  • R. Dengler, Hannover Medical School
  • ,
  • J. P. Camdessanché, Saint-Etienne University Hospital
  • ,
  • W. Nix, University Medical Center Johannes Gutenberg-University Mainz
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  • R. Liguori, Università di Bologna
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  • I. Schofield, Newcastle General Hospital
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  • L. Maderna, Universita degli Studi di Milano
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  • D. Czell, Neurology
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  • C. Neuwirth, Department of Radiology and Nuclear Medicine Kantonsspital St. Gallen
  • ,
  • M. Weber, Department of Radiology and Nuclear Medicine Kantonsspital St. Gallen
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  • V. E. Drory, Tel Aviv University
  • ,
  • A. Abraham, Tel Aviv University
  • ,
  • M. Swash, Queen Mary University of London
  • ,
  • M. de Carvalho, Instituto de Medicina Molecular

Objective: This study assesses inter-rater agreement and sensitivity of diagnostic criteria for amyotrophic lateral sclerosis (ALS). Methods: Clinical and electrophysiological data of 399 patients with suspected ALS were collected by eleven experienced physicians from ten different countries. Eight physicians classified patients independently and blinded according to the revised El Escorial Criteria (rEEC) and to the Awaji Criteria (AC). Inter-rater agreement was assessed by Kappa coefficients, sensitivity by majority diagnosis on 350 patients with follow-up data. Results: Inter-rater agreement was generally low both for rEEC and AC. Agreement was best on the categories “Not-ALS” “Definite” and “Probable” and poorest for “Possible” and “Probable Laboratory-supported”. Sensitivity was equal for rEEC (64%) and AC (63%), probably due to downgrading of “Probable Laboratory-supported” patients by AC. However, AC was significantly more effective in classifying patients as “ALS” versus “Not-ALS” (p < 0.0001). Conclusions: Inter-rater variation is high both for rEEC and for AC probably due to a high complexity of the rEEC inherent in the AC. The gain of AC on diagnostic sensitivity is reduced by the omission of the “Probable Laboratory-supported” category. Significance: The results highlight a need for initiatives to develop simpler and more reproducible diagnostic criteria for ALS in clinical practice and research.

Original languageEnglish
JournalClinical Neurophysiology
Pages (from-to)307-314
Number of pages8
Publication statusPublished - Feb 2019

    Research areas

  • Amyotrophic lateral sclerosis, Awaji criteria, Diagnostic criteria, Electrodiagnosis, Electromyography, Inter-rater variation, Revised El Escorial criteria

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