OBJECTIVE: To estimate the potential diagnostic added value of the Awaji 
criteria for diagnosis of a myotrophiclateral sclerosis (ALS), which have been 
compared with the previously accepted gold standard the revised El Escorial 
criteria in several studies.
DATA SOURCES: MEDLINE and Web of Science (until October2011).
STUDY SELECTION: We searched for studies testing the diagnostic accuracy of the 
Awaji criteria vs the revised El Escorial criteria in patients referred with 
suspected ALS.
DATA EXTRACTION: Evaluation and data extraction of identified studies were done 
independently. The Quality Assessment of Diagnostic Accuracy Studies list was 
used to assess study quality. We determined the proportion of patients 
classified as having probable/definite ALS and derived indices of diagnostic 
performance(sensitivity, specificity, and diagnostic odds ratio). Quantitative 
data synthesis was accomplished through random-effects meta-analysis, and 
heterogeneity was assessed with the I2 test.
DATA SYNTHESIS: Eight studies were included (3 prospective and 5 retrospective) 
enrolling 1187 patients. Application of Awaji criteria led to a 23% (95% CI, 12% 
to 33%; I2=84%) increase in the proportion of patients classified as having 
probable/definite ALS. Diagnostic performance of the Awaji criteria was higher 
than the revised El Escorial criteria (pooled sensitivity: 81.1% [95%CI, 72.2% 
to 90.0%; I2=91%] vs 62.2% [95% CI, 49.4%to 75.1%; I2=93%]; pooled diagnostic 
odds ratio, 35.8[95% CI, 15.2 to 84.7; I2=3%] vs 8.7 [95% CI, 2.2 to 
35.6;I2=50%]). Diagnostic accuracy of Awaji criteria was higher in bulbar- than 
in limb-onset cases.
CONCLUSION: The Awaji criteria have a significant clinical impact allowing 
earlier diagnosis and clinical trial entry in ALS.