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

Research output: Contribution to journalArticle

Abstract

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 : official journal of the International Federation of Clinical Neurophysiology
Volume130
Issue number2
DOIs
Publication statusPublished - Feb 2019

Fingerprint

Amyotrophic Lateral Sclerosis
Multicenter Studies
Physicians
Research

Cite this

Diagnostic criteria for amyotrophic lateral sclerosis : A multicentre study of inter-rater variation and sensitivity. / Johnsen, B; Pugdahl, K; Fuglsang-Frederiksen, A; Kollewe, K; Paracka, L; Dengler, R; Camdessanché, J P; Nix, W; Liguori, R; Schofield, I; Maderna, L; Czell, D; Neuwirth, C; Weber, M; Drory, V E; Abraham, A; Swash, M; de Carvalho, M.

In: Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology, Vol. 130, No. 2, 02.2019, p. 307-314.

Research output: Contribution to journalArticle

Johnsen, B, Pugdahl, K, Fuglsang-Frederiksen, A, Kollewe, K, Paracka, L, Dengler, R, Camdessanché, JP, Nix, W, Liguori, R, Schofield, I, Maderna, L, Czell, D, Neuwirth, C, Weber, M, Drory, VE, Abraham, A, Swash, M & de Carvalho, M 2019, 'Diagnostic criteria for amyotrophic lateral sclerosis: A multicentre study of inter-rater variation and sensitivity', Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology, vol. 130, no. 2, pp. 307-314. https://doi.org/10.1016/j.clinph.2018.11.021
Johnsen, B ; Pugdahl, K ; Fuglsang-Frederiksen, A ; Kollewe, K ; Paracka, L ; Dengler, R ; Camdessanché, J P ; Nix, W ; Liguori, R ; Schofield, I ; Maderna, L ; Czell, D ; Neuwirth, C ; Weber, M ; Drory, V E ; Abraham, A ; Swash, M ; de Carvalho, M. / Diagnostic criteria for amyotrophic lateral sclerosis : A multicentre study of inter-rater variation and sensitivity. In: Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology. 2019 ; Vol. 130, No. 2. pp. 307-314.
@article{ed2dbdc4555c4ee1b4fcacecae32c1e0,
title = "Diagnostic criteria for amyotrophic lateral sclerosis: A multicentre study of inter-rater variation and sensitivity",
abstract = "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.",
author = "B Johnsen and K Pugdahl and A Fuglsang-Frederiksen and K Kollewe and L Paracka and R Dengler and Camdessanch{\'e}, {J P} and W Nix and R Liguori and I Schofield and L Maderna and D Czell and C Neuwirth and M Weber and Drory, {V E} and A Abraham and M Swash and {de Carvalho}, M",
note = "Copyright {\circledC} 2018 International Federation of Clinical Neurophysiology. All rights reserved.",
year = "2019",
month = "2",
doi = "10.1016/j.clinph.2018.11.021",
language = "English",
volume = "130",
pages = "307--314",
journal = "Clinical Neurophysiology",
issn = "1388-2457",
publisher = "Elsevier Ireland Ltd",
number = "2",

}

TY - JOUR

T1 - Diagnostic criteria for amyotrophic lateral sclerosis

T2 - A multicentre study of inter-rater variation and sensitivity

AU - Johnsen, B

AU - Pugdahl, K

AU - Fuglsang-Frederiksen, A

AU - Kollewe, K

AU - Paracka, L

AU - Dengler, R

AU - Camdessanché, J P

AU - Nix, W

AU - Liguori, R

AU - Schofield, I

AU - Maderna, L

AU - Czell, D

AU - Neuwirth, C

AU - Weber, M

AU - Drory, V E

AU - Abraham, A

AU - Swash, M

AU - de Carvalho, M

N1 - Copyright © 2018 International Federation of Clinical Neurophysiology. All rights reserved.

PY - 2019/2

Y1 - 2019/2

N2 - 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.

AB - 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.

U2 - 10.1016/j.clinph.2018.11.021

DO - 10.1016/j.clinph.2018.11.021

M3 - Article

VL - 130

SP - 307

EP - 314

JO - Clinical Neurophysiology

JF - Clinical Neurophysiology

SN - 1388-2457

IS - 2

ER -