B Johnsen1, K Pugdahl2, A Fuglsang-Frederiksen2, K Kollewe3, L Paracka3, R Dengler3, J P Camdessanché4, W Nix5, R Liguori6, I Schofield7, L Maderna8, D Czell9, C Neuwirth10, M Weber10, V E Drory11, A Abraham11, M Swash12, M de Carvalho13. 1. Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark. Electronic address: birgjohn@rm.dk. 2. Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark. 3. Department of Neurology, Hannover Medical School, Hannover, Germany. 4. Department of Neurology, Saint-Etienne University Hospital, Saint-Etienne, France. 5. Department of Neurology, University Clinics Mainz, Mainz, Germany. 6. IRCCS Institute of Neurological Sciences of Bologna and Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy. 7. Department of Clinical Neurophysiology, Newcastle General Hospital, Newcastle upon Tyne, UK. 8. Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, University of Milano, Milan, Italy. 9. Neurology, Spital Linth, Uznach, Switzerland. 10. Neuromuscular Diseases Unit/ALS Clinic, Cantonal Hospital St. Gallen, St. Gallen, Switzerland. 11. Neuromuscular Service of the Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel. 12. Barts and the London School of Medicine, Queen Mary University of London, London, UK. 13. Institute of Physiology-Instituto de Medicina Molecular, Faculty of Medicine, University of Lisbon, and Department of Neurosciences, Hospital de Santa Maria-CHLN, Lisbon, Portugal.
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.
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.
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