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

Abstract

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.

Keywords:

• Amyotrophic lateral sclerosis
• frontotemporal degeneration
• cognitive impairment
• Edinburgh Cognitive and Behavioral ALS Screen
• diagnostic

Author contributions

ENA and BI: conceptualization, statistical analyses, draft, revision. DP and AR: conceptualization, revision. LP and FP: data collection, draft, revision. AC, CM, AC, GM and AC: conceptualization, resources, draft, revision.

Ethical approval

This study received ethical approval by the Comitato Etico Azienda Ospedaliero Universitaria Città della Salute e della Scienza (I.D. 00263/2020). Patients provided informed consent and data were treated according to current Italian regulations.

Declaration of interest

Edoardo Nicolò Aiello, Barbara Iazzolino, Debora Pain, Laura Peotta, Francesca Palumbo, Alice Radici, Antonio Canosa, Cristina Moglia, Gabriele Mora report no conflicts of interest. Andrea Calvo has received a research grant from Cytokinetics. Adriano Chiò serves on scientific advisory boards for Mitsubishi Tanabe, Roche, Biogen, Denali Pharma, Amylyx, and Cytokinetics. The sponsor organizations had no role in data collections and analysis and did not participate to writing and approving the manuscript. The information reported in the manuscript has never been reported elsewhere.

Additional information

Funding

The authors would like to thank patients and their relatives, together with the other volunteers who participated to this research. This work was in part supported by the Italian Ministry of Health (Ministero della Salute, Ricerca Sanitaria Finalizzata, grant RF-2016-02362405), the European Commission’s Health Seventh Framework Programme (FP7/2007-2013 under grant agreement 259867), and the Joint Programme - Neurodegenerative Disease Research (Strength, ALS-Care and Brain-Mend projects), granted by Italian Ministry of Education, University and Research. This study was in part supported by a Thierry Latran foundation grant (INSPIRED). This study was performed under the Department of Excellence grant of the Italian Ministry of Education, University and Research to the ‘Rita Levi Montalcini’ Department of Neuroscience, University of Torino, Italy.