Awaji Criteria for the Diagnosis of Amyotrophic Lateral Sclerosis A Systematic Review

Objective: To estimate the potential diagnostic added value of the Awaji criteria for diagnosis of amyotrophic lateral 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 October 2011). 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 I-2 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%; I-2=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%; I-2=91%] vs 62.2% [95% CI, 49.4% to 75.1%; I-2=93%]; pooled diagnostic odds ratio, 35.8 [95% CI, 15.2 to 84.7; I-2=3%] vs 8.7 [95% CI, 2.2 to 35.6; I-2=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.