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Clinimetrics of the cognitive section of the Italian ALS Cognitive Behavioral Screen (ALS-CBS™)

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Abstract

Background

The present study aimed at (1) providing further validity and reliability evidence for the Italian version of the cognitive section of the ALS Cognitive Behavioral Screen (ALS-CBS™) and (2) testing its diagnostics within an Italian ALS cohort, as well as at (3) exploring its capability to discriminate patients from healthy controls (HCs).

Methods

N = 293 non-demented ALS patients were administered the cognitive sections of the ALS-CBS™ and Edinburgh Cognitive and Behavioural ALS Screen (ECAS). N = 96 HCs demographically matched with N = 96 patients were also administered the cognitive section of the ALS-CBS™. In patients, factorial and construct validity, internal reliability, and diagnostics against a defective score on the cognitive section of the ECAS were tested. Case–control discrimination was assessed via a logistic regression.

Results

ALS-CBS™ cognitive subscales were underpinned by a simple, unidimensional structure, internally reliable (McDonald’s ω = 0.74), and mostly related with ECAS executive and fluency scores (rs = 0.54–0.71). Both raw and age- and education-adjusted scores on the cognitive section of the ALS-CBS™ accurately detected ECAS-defined cognitive impairment (AUC = 0.80 and .88, respectively), yielding optimal error-based, information-based and unitary diagnostics. A cut-off of < 15.374 was identified on adjusted scores. The test was able to discriminate patients from HCs (p < 0.001).

Discussion

The cognitive section of the Italian ALS-CBS™ is a valid, reliable, and diagnostically sound ALS-specific screener for detecting frontotemporal, executive-/attentive-based cognitive inefficiency in non-demented ALS patients, being also able to discriminate them from normotypical individuals.

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Data availability

Datasets associated with the present study are available upon reasonable request of interested researchers.

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Acknowledgements

The authors are thankful to patients and their caregivers, as well as to healthy participants.

Funding

This research was funded by the Italian Ministry of Health to IRCCS Istituto Auxologico Italiano (Ricerca Corrente, project 23C302).

Author information

Author notes

  1. Edoardo Nicolò Aiello and Lucia Catherine Greco contributed equally; Valeria Ada Sansone and Barbara Poletti contributed equally as well.

Authors and Affiliations

  1. Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149, Milan, Italy

    Edoardo Nicolò Aiello, Federica Solca, Silvia Torre, Laura Carelli, Federico Verde, Vincenzo Silani, Nicola Ticozzi & Barbara Poletti

  2. PhD Program in Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy

    Edoardo Nicolò Aiello & Valeria Ada Sansone

  3. Neuromuscular Omnicentre (NEMO), Fondazione Serena Onlus, Milan, Italy

    Lucia Catherine Greco, Andrea Lizio, Jacopo Casiraghi & Federica Cerri

  4. NeMO Lab, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy

    Lucia Catherine Greco

  5. Department of Human and Social Sciences, University of Bergamo, Bergamo, Italy

    Antonino La Tona, Agostino Brugnera & Angelo Compare

  6. Istituti Clinici Scientifici Maugeri IRCCS, Neurorehabilitation Department of Milan Institute, Milan, Italy

    Debora Pain, Alice Radici & Christian Lunetta

  7. Syneos Health, Morrisville, NC, USA

    Susan Woolley

  8. Biogen, Cambridge, MA, USA

    Jennifer Murphy

  9. School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy

    Lucio Tremolizzo & Ildebrando Appollonio

  10. Department of Pathophysiology and Transplantation, Dino Ferrari Center”, Università degli Studi di Milano, Milan, Italy

    Federico Verde, Vincenzo Silani & Nicola Ticozzi

  11. Department of Biomedical Sciences of Health, University of Milan, Milan, Italy

    Valeria Ada Sansone

Authors
  1. Edoardo Nicolò Aiello
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  2. Lucia Catherine Greco
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  3. Antonino La Tona
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  4. Federica Solca
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  6. Laura Carelli
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  8. Alice Radici
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  9. Andrea Lizio
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  13. Angelo Compare
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  14. Susan Woolley
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  15. Jennifer Murphy
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  19. Vincenzo Silani
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  21. Christian Lunetta
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  22. Valeria Ada Sansone
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  23. Barbara Poletti
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Corresponding author

Correspondence to Barbara Poletti.

Ethics declarations

Ethics approval and informed consent

Participants provided informed consent and data were treated according to current regulations. This study was approved by the Ethics Committee of IRCCS Istituto Auxologico Italiano (I.D.: 2013_06_25), by the Ethics Committee of Milano Niguarda Area 3 (I.D.: 393–09062021) and by the Ethics Committee of Istituti Clinici Scientifici Maugeri IRCCS (I.D.: CE 2495—12012021).

Conflict of interest

V. S. received compensation for consulting services and/or speaking activities from AveXis, Cytokinetics, Italfarmaco, Liquidweb S.r.l., and Novartis Pharma AG, receives or has received research supports from the Italian Ministry of Health, AriSLA, and E-Rare Joint Transnational Call. He is on the Editorial Board of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, European Neurology, American Journal of Neurodegenerative Diseases, Frontiers in Neurology. B.P. and L.C. received compensation for consulting services and/or speaking activities from Liquidweb S.r.l. B.P is Associate Editor for Frontier in Neuroscience. N. T. received compensation for consulting services from Amylyx Pharmaceuticals and Zambon Biotech SA. He is Associate Editor for Frontiers in Aging Neuroscience. C.L. received compensation for consulting services and/or speaking activities from Cytokinetics, Italfarmaco, and Mitsubishi Tanabe Pharma Europe. He is Associate Editor for Frontiers in Neurology. V.A.S. participates in Advisory Boards or teaching activities for Biogen, Roche, Avexis, PTC, Santhera, Sarepta, Dyne. S.W. receives licensing fees when the ALS Cognitive Behavioural Screen (ALS-CBS™) is used in pharmaceutical trials. J.M. is employed full time at Biogen; she does not receive compensations related to the ALS-CBS™. The remaining authors have nothing to declare.

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Aiello, E.N., Greco, L.C., La Tona, A. et al. Clinimetrics of the cognitive section of the Italian ALS Cognitive Behavioral Screen (ALS-CBS™). Neurol Sci 44, 1243–1249 (2023). https://doi.org/10.1007/s10072-022-06569-9

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