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A preliminary comparison between ECAS and ALS-CBS in classifying cognitive–behavioural phenotypes in a cohort of non-demented amyotrophic lateral sclerosis patients

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Abstract

To define the presence and type of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS), different screening tools have been created. Currently, the most used screening tests are the Edinburgh cognitive and behavioural ALS screen (ECAS) and the ALS cognitive behavioural screen (ALS-CBS). The objective of this study was to compare the ability of ECAS and ALS-CBS in classifying non-demented ALS patients according to Strong criteria. One-hundred and fifty-four in- and out-patients with an age > 18 and a definite or probable ALS diagnosis were recruited between September 2019 and February 2020 at NeMO Clinical Centre and at Istituto Auxologico Italiano in Milan and underwent the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and the ALS Cognitive Behavioural Screen (ALS-CBS). Exclusion criteria involved patients with a diagnosis of FTD, with a severe cognitive deterioration and/or an important behavioural impairment, with a significant psychiatric disorder or with the co-presence of another significant illness. The distribution of patients according to Strong criteria was different for ECAS and ALS-CBS and the degree of agreement between the two tests in terms of Cohen’s Kappa coefficient resulted equal to 0.2047 with a 95% confidence limits interval between 0.1122 and 0.2973. This study for the first time compares the ability of ECAS and ALS-CBS in stratifying ALS patients. Further studies will be conducted to better understand the reasons underlying the differences between these two tests in classifying the different subtypes of fronto-temporal dysfunction in ALS.

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Availability of data and materials

Data are available upon reasonable request. De-identified database will be shared upon reasonable request for 2 years after publication by contacting the corresponding author at the following e-mail: christian.lunetta@centrocliniconemo.it.

Code availability

The datasets generated and analyzed during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

We are grateful to all patients and caregivers that collaborated to the study.

Funding

This study received no funding.

Author information

Author notes

  1. Lucia Catherine Greco, Andrea Lizio, Barbara Poletti, and Christian Lunetta have contributed equally to the manuscript.

Authors and Affiliations

  1. Neuromuscular Omnicentre (NEMO), Fondazione Serena Onlus, Piazza Ospedale Maggiore, 3, 20162, Milan, Italy

    Lucia Catherine Greco, Andrea Lizio, Jacopo Casiraghi, Valeria Ada Sansone & Christian Lunetta

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

    Valeria Ada Sansone

  3. ALS Clinic, Neurology Unit, San Gerardo Hospital, University of Milano-Bicocca, Monza, Italy

    Lucio Tremolizzo

  4. Department of Neurology, Neurorehabilitation and Clinical Neurophysiology, IRCCS “San Raffaele Scientific Institute”, Milan, Italy

    Nilo Riva

  5. Neuropathology Unit, Division of Neuroscience, (IRCCS) San Raffaele Scientific Institute, Milan, Italy

    Nilo Riva

  6. Neuroimaging Research Unit, Division of Neuroscience, (IRCCS) San Raffaele Scientific Institute, Milan, Italy

    Massimo Filippi

  7. Vita-Salute San Raffaele University, Milan, Italy

    Massimo Filippi

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

    Federica Solca, Nicola Ticozzi & Vincenzo Silani

  9. Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy

    Silvia Torre, Nicola Ticozzi, Vincenzo Silani & Barbara Poletti

Authors
  1. Lucia Catherine Greco
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  2. Andrea Lizio
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  3. Jacopo Casiraghi
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  4. Valeria Ada Sansone
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  5. Lucio Tremolizzo
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  6. Nilo Riva
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  7. Federica Solca
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  8. Silvia Torre
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  9. Nicola Ticozzi
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  10. Massimo Filippi
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  11. Vincenzo Silani
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  12. Barbara Poletti
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  13. Christian Lunetta
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Contributions

CL and BP had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: CL, BP, LCG, and AL. Acquisition, analysis, or interpretation of data: LCG, ST, AL, FS, BP, and CL. Drafting of the manuscript: LCG and AL. Critical revision of the manuscript for important intellectual content: all authors. Statistical analysis: AL. Study supervision: CL and BP.

Corresponding author

Correspondence to Lucia Catherine Greco.

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Conflicts of interest

CL received compensation for occasional scientific consulting from Neuraltus, Cytokinetics, Mitsubishi Tanabe Pharma Europe and Italfarmaco and has received funds from ARISLA and Italian Ministry of Health, AL received compensation for occasional scientific consulting from Italfarmaco, VAS participates in Advisory Boards or Teaching activities for Biogen, Roche, Avexis, PTC, Santhera, and Sarepta, Dyne. Other authors declared no disclosures.

Ethics approval

The study design was approved by the institutional ethical committees of Milano Area 3 and all the subjects involved signed the informed consent in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Consent to participate

All the subjects involved in the study participated willingly.

Consent for publication

All the subjects involved signed the informed consent for publication data.

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Greco, L.C., Lizio, A., Casiraghi, J. et al. A preliminary comparison between ECAS and ALS-CBS in classifying cognitive–behavioural phenotypes in a cohort of non-demented amyotrophic lateral sclerosis patients. J Neurol 269, 1899–1904 (2022). https://doi.org/10.1007/s00415-021-10753-w

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  • DOI: https://doi.org/10.1007/s00415-021-10753-w

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