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ALS Cognitive Behavioral Screen (ALS-CBS): normative values for the Italian population and clinical usability

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Abstract

Amyotrophic lateral sclerosis (ALS) patients often express cognitive and behavioral dysfunctions within the so-called “frontotemporal spectrum disorders.” Guidelines recommend screening of such dysfunctions, albeit only ALS dedicated tools are eventually suitable, due to the profound motor limitations induced by the disease. ALS Cognitive Behavioral Screen (ALS-CBS) is such a screening tool but normative data are not available, limiting its widespread implementation. Our aim consisted in producing normative data for the Italian version of the ALS-CBS. The scale was administered to n = 458 healthy controls with different age and education. Following translation and back translation of the original version of the test, normative data and correction scores for the ALS-CBS cognitive subtest (ALS-CBSci) were generated. Furthermore, n = 100 ALS consecutive outpatients with a wide range of cognitive and motor severity underwent to the ALS-CBS, besides FAB and Weigl sorting test (WST), in order to check its usability. Completion rate was 100% for ALS-CBS and WST, and 68% for the FAB. Corrected ALS-CBS scores showed 12% detection rate of significant cognitive dysfunction with a moderate kappa with FAB and WST. For the ALS-CBS behavioral subtest (ALS-CBSbi), a caregiver was available for n = 81 ALS patients and asked to complete the subset. The detection rate for behavioral dysfunction was 55.5%, and a mild correlation between with the Caregiver Burden Inventory was present (r = − 0.26, p = 0.04). In conclusion, we offer here normative data for the ALS-CBS, a handy tool for screening frontotemporal spectrum dysfunctions in ALS patients, and confirm its usability and validity in an outpatient setting.

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Acknowledgments

Besides the authors, the ALS-CBS Italian Study Group includes the following collaborators: Angelo Aliprandi (Lecco), Diletta Cereda (Monza), Elisa Conti (Monza), Anna Maria Gallone (Monza), Vittorio Mantero (Lecco), Laura Marzorati (Monza), Lorena Mosca (Milano), Andrea Pellegrini (Monza), Michele Perini (Gallarate), Andrea Rigamonti (Lecco), Gessica Sala (Monza), Andrea Salmaggi (Lecco), Lucia Spinazzola (Gallarate), Emanuela Susani (Milano), Claudia Tarlarini (Milano), Francesca Trojsi (Napoli), Davide Zarcone (Gallarate); Italy.

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Authors and Affiliations

  1. School of Medicine and Surgery and Milan Center for Neuroscience (NeuroMI), University of Milano-Bicocca, U8 Via Cadore 48 –, 20900, Monza, MB, Italy

    Lucio Tremolizzo, Susanna Diamanti, Carlo Ferrarese & Ildebrando Appollonio

  2. Neurology, “San Gerardo” Hospital, Monza, Italy

    Lucio Tremolizzo, Susanna Diamanti, Carlo Ferrarese & Ildebrando Appollonio

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

    Andrea Lizio, Christian Lunetta, Francesca Gerardi & Valeria Ada Sansone

  4. Department of Psychology, University of Campania “Luigi Vanvitelli”, Viale Ellittico 31, 81100, Caserta, Italy

    Gabriella Santangelo

  5. NEuroMuscular Omnicenter (NEMO) SUD Clinical Center, Aurora Onlus Foundation, Messina, Italy

    Sonia Messina & Stefania La Foresta

  6. Institute of Experimental Neurology (INSPE), Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy

    Nilo Riva, Yuri Falzone & Massimo Filippi

  7. Sutter Pacific Medical Foundation, 5150 Hill Rd E, Lakeport, CA, 95453, USA

    Susan C. Woolley

  8. Department of Biomedical Sciences of Health, University of Milano, Via Festa del Perdono, 7, 20122, Milan, MI, Italy

    Valeria Ada Sansone

  9. Department of Advanced Medical and Surgical Sciences - MRI Research Center Vanvitelli-FISM, University of Campania “Luigi Vanvitelli”, Naples, Italy

    Mattia Siciliano

Authors
  1. Lucio Tremolizzo
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  2. Andrea Lizio
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  3. Gabriella Santangelo
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  5. Christian Lunetta
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  6. Francesca Gerardi
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  7. Sonia Messina
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  9. Nilo Riva
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  12. Susan C. Woolley
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  14. Mattia Siciliano
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  15. Carlo Ferrarese
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  16. Ildebrando Appollonio
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Consortia

for the ALS-CBS Italian Study Group

  • Angelo Aliprandi
  • , Diletta Cereda
  • , Elisa Conti
  • , Anna Maria Gallone
  • , Vittorio Mantero
  • , Laura Marzorati
  • , Lorena Mosca
  • , Andrea Pellegrini
  • , Michele Perini
  • , Andrea Rigamonti
  • , Gessica Sala
  • , Andrea Salmaggi
  • , Lucia Spinazzola
  • , Emanuela Susani
  • , Claudia Tarlarini
  • , Francesca Trojsi
  •  & Davide Zarcone

Corresponding authors

Correspondence to Lucio Tremolizzo or Gabriella Santangelo.

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No conflicts of interest were reported by all the Authors.

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This study has been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

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Subjects were recruited following ethical approval and informed consent.

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Tremolizzo, L., Lizio, A., Santangelo, G. et al. ALS Cognitive Behavioral Screen (ALS-CBS): normative values for the Italian population and clinical usability. Neurol Sci 41, 835–841 (2020). https://doi.org/10.1007/s10072-019-04154-1

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  • DOI: https://doi.org/10.1007/s10072-019-04154-1

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