OBJECTIVE: To assess the sensitivity and specificity of the Awaji and revised El 
Escorial diagnostic criteria (rEEC) in amyotrophic lateral sclerosis (ALS).
METHODS: We conducted a large prospective multicenter study, recruiting 416 
patients (253 male, 163 female) between January 1, 2012, and August 31, 2015, to 
compare the diagnostic accuracy of Awaji and rEEC in accordance with standards 
of reporting of diagnostic accuracy criteria.
RESULTS: The sensitivity of the Awaji criteria (57%, 50.0%-63.3%) was higher 
when compared to rEEC (45%, 38.7%-51.7%, p < 0.001), translating to a 12% gain 
in sensitivity. The specificity of the both criteria were identical, 99.5%, 
indicating the number needed to test in order to diagnose one extra case of ALS 
was 1.8 (1.5-2) for Awaji criteria and 2.4 (2-2.6) for rEEC. The Awaji criteria 
exhibited a higher sensitivity across subgroups, including bulbar (p < 0.001) 
and limb-onset (p < 0.001) patients. The inclusion of the possible diagnostic 
category as a positive finding enhanced sensitivity of the Awaji criteria and 
rEEC, particularly in early stages of ALS, while maintaining specificity.
CONCLUSION: The present study established a higher sensitivity of Awaji criteria 
when compared to rEEC, with diagnostic benefits evident in bulbar and limb-onset 
disease. Inclusion of possible as a positive finding enhanced sensitivity of 
both criteria, while maintaining specificity, and should be considered in 
clinical practice and future therapeutic trials.
CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that the Awaji 
criteria have a higher sensitivity and the same specificity as the rEEC in 
identifying patients with ALS.