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Ecological validity of performance-based cognitive screeners in amyotrophic lateral sclerosis: preliminary evidence

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Abstract

Background

This study aimed at preliminarily assessing, in a cohort of non-demented amyotrophic lateral sclerosis (ALS) patients, the ecological validity, and more specifically the veridicality, of the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and the ALS Cognitive Behavioral Screen (ALS-CBS™), by relating their scores to caregiver-report ratings of cognitive changes.

Methods

N = 147 patient-caregiver dyads were recruited. Patients were administered the ECAS and ALS-CBS™, whilst caregiver the Caregiver Behavioral Questionnaire (CBQ) and Beaumont Behavioural Inventory (BBI). An Ecological Cognitive Functioning Index (ECFI) was derived from those items of the CBQ and BBI that tap on executive and language changes. Ecological validity was assessed via both correlational and predictive analyses net of caregiver-rated behavioural changes (as assessed by the ECAS-Carer Interview).

Results

The ECFI was associated with the total scores on both the ECAS (p = .014) and ALS-CBS™ (p = .017). When looking at ECAS and ALS-CBS™ subscales, those assessing verbal fluency were selectively associated with the ECFI. The ECFI was higher in patients performing defectively on the ECAS (p = .004) and on the ALS-CBS™ (p = .027).

Discussion

This study suggests that both the ECAS and the ALS-CBS™ represent a valid estimate of non-demented ALS patients’ cognitive status in the real world, also highlighting the clinical relevance of cognitive changes reported by caregivers.

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Data availability

Datasets associated with the present study have been stored on an online repository (https://doi.org/10.5281/zenodo.12179998). These datasets cannot be made publicly available on ethical-legal grounds but can be made available upon reasonable request of interested researchers to the corresponding author, who will forward a request for a data transfer agreement to the relevant Ethical Committee.

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Acknowledgements

the Authors are thankful to patients and their caregivers. The Authors acknowledge the support of the Italian Ministry of Health – Ricerca Corrente and of the Italian Ministry of Education and Research (“Dipartimenti di Eccellenza” Program 2023–2027 - Department of Pathophysiology and Transplantation, “Dino Ferrari” Center, Università degli Studi di Milano).

Funding

This work was supported by Italian Ministry of Health - Ricerca Finalizzata (RF-2021-12374238).

Author information

Authors and Affiliations

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

    Edoardo Nicolò Aiello, Silvia Torre, Federica Solca, Beatrice Curti, Giulia De Luca, Eleonora Colombo, Alessio Maranzano, Federico Verde, Claudia Morelli, Stefano Messina, Alberto Doretti, Vincenzo Silani, Nicola Ticozzi & Barbara Poletti

  2. Neurology Residency Program, Università degli Studi di Milano, Milano, Italy

    Claudia Gendarini & Alessandro Cocuzza

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

    Federico Verde, Vincenzo Silani & Nicola Ticozzi

  4. Department of Oncology and Hemato-Oncology, Università degli Studi di Milano, Milano, Italy

    Barbara Poletti

Authors
  1. Edoardo Nicolò Aiello
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  2. Silvia Torre
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  3. Federica Solca
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Contributions

ENA: conceptualization, analyses, drafting, revision; FS, BC, GDL: analyses, drafting, revision; CG, AC, EC, AM: data collection, revision; CM, SM, AD, FV, VS: resources, revision; BP, NT: resources, drafting, revision.

Corresponding author

Correspondence to Barbara Poletti.

Ethics declarations

Ethical approval

Participants provided informed consent. This study was approved by the Ethics Committee of IRCCS Istituto Auxologico Italiano (I.D.: 2013_06_25).

Disclosure

V. S. received compensation for consulting services and/or speaking activities from Ave**s, 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 in the Editorial Board of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, European Neurology, American Journal of Neurodegenerative Diseases, Frontiers in Neurology. B.P. received compensation for consulting services and/or speaking activities from Liquidweb S.r.l. She 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. E.N.A. serves as an Editorial Board Member for BMC Neurology. F.V. is Associated Editor for Journal of Alzheimer’s Disease.

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Aiello, E.N., Torre, S., Solca, F. et al. Ecological validity of performance-based cognitive screeners in amyotrophic lateral sclerosis: preliminary evidence. Neurol Sci (2024). https://doi.org/10.1007/s10072-024-07660-z

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