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01-04-2020 | Amyotrophic Lateral Sclerosis | Original Article

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

Authors: Lucio Tremolizzo, Andrea Lizio, Gabriella Santangelo, Susanna Diamanti, Christian Lunetta, Francesca Gerardi, Sonia Messina, Stefania La Foresta, Nilo Riva, Yuri Falzone, Massimo Filippi, Susan C. Woolley, Valeria Ada Sansone, Mattia Siciliano, Carlo Ferrarese, Ildebrando Appollonio, for the ALS-CBS Italian Study Group

Published in: Neurological Sciences | Issue 4/2020

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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Woolley SC, Rush BK (2017) Considerations for clinical neuropsychological evaluation in amyotrophic lateral sclerosis. Arch Clin NeuropsycholArch Clin Neuropsychol 32:906–916CrossRef

Montuschi A, Iazzolino B, Calvo A, Moglia C, Lopiano L, Restagno G, Brunetti M, Ossola I, Lo Presti A, Cammarosano S, Canosa A, Chiò A (2015) Cognitive correlates in amyotrophic lateral sclerosis: a population-based study in Italy. J Neurol Neurosurg PsychiatryJ Neurol Neurosurg Psychiatry 86:168–173CrossRef

Murphy J, Factor-Litvak P, Goetz R, Lomen-Hoerth C, Nagy PL, Hupf J, Singleton J, Woolley S, Andrews H, Heitzman D, Bedlack RS, Katz JS, Barohn RJ, Sorenson EJ, Oskarsson B, Fernandes Filho JA, Kasarskis EJ, Mozaffar T, Rollins YD, Nations SP, Swenson AJ, Koczon-Jaremko BA, Mitsumoto H, ALS COSMOS (2016) Cognitive-behavioral screening reveals prevalent impairment in a large multicenter ALS cohort. Neurology. 86:813–820CrossRef

Chiò A, Vignola A, Mastro E, Giudici AD, Iazzolino B, Calvo A, Moglia C, Montuschi A (2010) Neurobehavioral symptoms in ALS are negatively related to caregivers’ burden and quality of life. Eur J NeurolEur J Neurol 17:1298–1303CrossRef

Chiò A, Ilardi A, Cammarosano S, Moglia C, Montuschi A, Calvo A (2012) Neurobehavioral dysfunction in ALS has a negative effect on outcome and use of PEG and NIV. Neurology. 78:1085–1089CrossRef

Strong MJ, Abrahams S, Goldstein LH, Woolley S, Mclaughlin P, Snowden J, Mioshi E, Roberts-South A, Benatar M, HortobáGyi T, Rosenfeld J, Silani V, Ince PG, Turner MR (2017) Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): revised diagnostic criteria. Amyotroph Lateral Scler Frontotemporal Degener 18:153–174CrossRef

Tremolizzo L, Ferrarese C, Appollonio I (2013) Exploring limits of neuropsychological screening in ALS: the FAB problem. Amyotroph Lateral Scler Frontotemporal Degener 14:157–158CrossRef

Poletti B, Solca F, Carelli L, Madotto F, Lafronza A, Faini A, Monti A, Zago S, Calini D, Tiloca C, Doretti A, Verde F, Ratti A, Ticozzi N, Abrahams S, Silani V (2016) The validation of the Italian Edinburgh cognitive and behavioural ALS Screen (ECAS) (2016). Amyotroph Lateral Scler Frontotemporal Degener 17:489–498CrossRef

Siciliano M, Trojano L, Trojsi F, Greco R, Santoro M, Basile G, Piscopo F, D’Iorio A, Patrone M, Femiano C, Monsurrò M, Tedeschi G, Santangelo G (2017) Edinburgh cognitive and behavioural ALS screen (ECAS)-Italian version: regression based norms and equivalent scores. Neurol SciNeurol Sci 38:1059–1068CrossRef

10.

Woolley SC, York MK, Moore DH, Strutt AM, Murphy J, Schulz PE, Katz JS (2010) Detecting frontotemporal dysfunction in ALS: utility of the ALS cognitive behavioral screen (ALS-CBS). Amyotroph Lateral SclerAmyotroph Lateral Scler 11:303–311CrossRef

11.

Cohen JW (1988) Statistical power analysis for the behavioral sciences, 2nd edn. Lawrence Erlbaum Associates Publishers, Hillsdale

12.

Brugnolo A, De Carli F, Accardo J, Amore M, Bosia LE, Bruzzaniti C, Cappa SF, Cocito L, Colazzo G, Ferrara M, Ghio L, Magi E, Mancardi GL, Nobili F, Pardini M, Rissotto R, Serrati C, Girtler N (2016) An updated Italian normative dataset for the Stroop color word test (SCWT). Neurol Sci 37:365–372CrossRef

13.

Measso G, Cavarzeran F, Zappalà G, Lebowitz BD, Crook TH, Pirozzolo FJ, Amaducci LA, Massari D, Grigoletto F (1993) The mini-mental state examination: normative study of an Italian random sample. Dev NeuropsycholDev Neuropsychol 9:77–85CrossRef

14.

Appollonio I, Leone M, Isella V, Piamarta F, Consoli T, Villa ML, Forapani E, Russo A, Nichelli P (2005) The frontal assessment battery (FAB): normative values in an Italian population sample. Neurol SciNeurol Sci 26:108–116CrossRef

15.

Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseasese (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299CrossRef

16.

Laiacona M, Inzaghi MG, De Tanti A, Capitani E (2000) Wisconsin card sorting test: a new global score, with Italian norms, and its relationship with the Weigl sorting test. Neurol SciNeurol Sci 21:279–291CrossRef

17.

Novak M, Guest C (1989) Application of a multidimensional caregiver burden inventory. Gerontologist. 29:798–803CrossRef

18.

Spinnler H, Tognoni G (1987) Italian standardization and adjustment of neuropsychological tests. Ital J Neurol SciItal J Neurol Sci 6(Suppl.8):8–20

19.

Tremolizzo L, Pellegrini A, Susani E, Lunetta C, Woolley SC, Ferrarese C, Appollonio I (2016) Behavioural but not cognitive impairment is a determinant of caregiver burden in amyotrophic lateral sclerosis. Eur NeurolEur Neurol 75:191–194CrossRef

20.

Floris G, Borghero G, Chiò A, Secchi L, Cannas A, Sardu C, Calvo A, Moglia C, Marrosu MG (2012) Cognitive screening in patients with amyotrophic lateral sclerosis in early stages. Amyotroph Lateral SclerAmyotroph Lateral Scler 13:95–101CrossRef

21.

Elamin M, Phukan J, Bede P, Jordan N, Byrne S, Pender N, Hardiman O (2011) Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology. 76:1263–1269CrossRef

22.

Trojsi F, Siciliano M, Femiano C, Santangelo G, Lunetta C, Calvo A, Moglia C, Marinou K, Ticozzi N, Drago Ferrante G, Scialò C, Sorarù G, Conte A, Falzone YM, Tortelli R, Russo M, Sansone VA, Chiò A, Mora G, Poletti B, Volanti P, Caponnetto C, Querin G, Sabatelli M, Riva N, Logroscino G, Messina S, Fasano A, Monsurrò MR, Tedeschi G, Mandrioli J (2017) Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort. J NeurolJ Neurol 264:2224–2231CrossRef

23.

Turon-Sans J, Gascon-Bayarri J, Reñé R, Rico I, Gámez C, Paipa A, Povedano M (2016) Cognitive impairment in ALS patients and validation of the Spanish version of the ALS-CBS test. Amyotroph Lateral Scler Frontotemporal Degener 17:221–227CrossRef

24.

Branco LM, Zanao T, De Rezende TJ, Casseb RF, Balthazar MF, Woolley SC, França MC Jr (2017) Transcultural validation of the ALS-CBS cognitive section for the Brazilian population. Amyotroph Lateral Scler Frontotemporal Degener 18:60–67CrossRef

25.

Christodoulou G, Gennings C, Hupf J, Factor-Litvak P, Murphy J, Goetz RR, Mitsumoto H (2016) Telephone based cognitive-behavioral screening for frontotemporal changes in patients with amyotrophic lateral sclerosis (ALS). Amyotroph Lateral Scler Frontotemporal Degener 17:482–488CrossRef

26.

ISTAT, 2015: Istituto Nazionale di Statistica (2015) [Italy’s National Statistics Institute (2015)]. Rapporto Annuale. La situazione del paese nel 2015 [Annual report: the state of the nation in 2015]. Italy

27.

Palmieri A, Mento G, Calvo V, Querin G, D’Ascenzo C, Volpato C, Kleinbub JR, Bisiacchi PS, Sorarù G (2015) Female gender doubles executive dysfunction risk in ALS: a case-control study in 165 patients. J Neurol Neurosurg PsychiatryJ Neurol Neurosurg Psychiatry 86:574–579CrossRef

28.

Crockford C, Newton J, Lonergan K, Madden C, Mays I, O’Sullivan M, Costello E, Pinto-Grau M, Vajda A, Heverin M, Pender N, Al-Chalabi A, Hardiman O, Abrahams S (2018) Measuring reliable change in cognition using the Edinburgh cognitive and behavioural ALS screen (ECAS). Amyotroph Lateral Scler Frontotemporal Degener 19:65–73CrossRef

29.

Poletti B, Solca F, Carelli L, Faini A, Madotto F, Lafronza A, Monti A, Zago S, Ciammola A, Ratti A, Ticozzi N, Abrahams S, Silani V (2018) Cognitive-behavioral longitudinal assessment in ALS: the Italian Edinburgh cognitive and behavioral ALS screen (ECAS). Amyotroph Lateral Scler Frontotemporal Degener 19:387–395CrossRef

Title: ALS Cognitive Behavioral Screen (ALS-CBS): normative values for the Italian population and clinical usability
Authors: Lucio Tremolizzo
Andrea Lizio
Gabriella Santangelo
Susanna Diamanti
Christian Lunetta
Francesca Gerardi
Sonia Messina
Stefania La Foresta
Nilo Riva
Yuri Falzone
Massimo Filippi
Susan C. Woolley
Valeria Ada Sansone
Mattia Siciliano
Carlo Ferrarese
Ildebrando Appollonio
for the ALS-CBS Italian Study Group
Publication date: 01-04-2020
Publisher: Springer International Publishing
Keyword: Amyotrophic Lateral Sclerosis
Published in: Neurological Sciences / Issue 4/2020
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI: https://doi.org/10.1007/s10072-019-04154-1

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

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