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

B. Johnsen*, K. Pugdahl, A. Fuglsang-Frederiksen, K. Kollewe, L. Paracka, R. Dengler, J. P. Camdessanché, W. Nix, R. Liguori, I. Schofield, L. Maderna, D. Czell, C. Neuwirth, M. Weber, V. E. Drory, A. Abraham, M. Swash, M. de Carvalho

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


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
Pages (from-to)307-314
Number of pages8
JournalClinical Neurophysiology
Issue number2
StatePublished - Feb 2019


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


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