The diagnostic value of the Italian version of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS)

Objectives: The diagnostic capabilities of specific cognitive screeners are clinically crucial for an early detection of frontotemporal features in amyotrophic lateral sclerosis (ALS) patients. This study aimed at providing diagnostic properties of the cognitive section of the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) in Italian ALS patients. Methods: Eighty-nine consecutive Italian ALS patients were classified according to Strong et al. (2017) criteria with a comprehensive neuropsychological assessment. The Italian version of ECAS was also administered, and its accuracy, sensitivity (SE), specificity (SP), negative and positive predictive values (PPV; NPV) and likelihood ratios (LR+; LR-) were computed against clinical diagnoses. Results: The ECAS and its subscales yielded moderate-to-high accuracy (AUC =.7–.87). High SP was overall found (81.8%–100%). The most sensitive measures were ALS-specific and Executive (73.9–78.3%) subscales, while the least were the ALS-non-specific subscales (8.7–17.4%). ECAS measures showed good PPVs (60%–100%) and NPVs (75.9%–91.5%). Acceptable LRs were found (LR+: 6.97–4.3; LR–:.29–.91), with total and ALS-specific measures yielding the best estimates. Conclusions: This is the first study demonstrating the diagnostic value of the ECAS against a thorough neuropsychological assessment in Italy. ECAS-total and -ALS-specific scores are diagnostically sound as to both intrinsic and post-test features, whereas ALS-non-specific measures perform slightly worse.