Abstract
Cognitive assessment for individuals with Amyotrophic Lateral Sclerosis (ALS) can be difficult because of frequent occurrence of difficulties with speech, writing, and drawing. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) is a recent multi-domain neuropsychological screening tool specifically devised for this purpose, and it assesses the following domains: executive functions, social cognition, verbal fluency and language (ALS-specific), but also memory and visuospatial abilities (Non-ALS specific). ECAS total score ranges from 0 (worst performance) to 136 (best performance). Moreover, a brief caregiver interview provides an assessment of behaviour changes and psychotic symptoms usually associated with ALS patients. The aim of the present study was to provide normative values for ECAS total score and sub-scores in a sample of Italian healthy subjects. Two hundred and seventy-seven Italian healthy subjects (151 women and 126 men; age range 30–79 years; educational level from primary school to university) underwent ECAS and Montreal Cognitive Assessment (MoCA). Multiple linear regression analysis revealed that age and education significantly influenced performance on ECAS total score and sub-scale scores. From the derived linear equation, a correction grid for raw scores was built. Inferential cut-off scores were estimated using a non-parametric technique and equivalent scores (ES) were computed. Correlation analysis showed a good significant correlation between adjusted ECAS total scores with adjusted MoCA total scores (r rho = 0.669, p < 0.0001). The present study provided normative data for the ECAS in an Italian population useful for both clinical and research purposes.
Similar content being viewed by others
References
Goldstein LH, Abrahams S (2013) Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment. Lancet Neurol 12:368–380. doi:10.1016/S1474-4422(13)70026-7
Abrahams S, Leigh PN, Harvey A, Vythelingum GN, Grisé D, Goldstein LH (2000) Verbal fluency and executive dysfunction in amyotrophic lateral sclerosis (ALS). Neuropsychologia 38:734–747. doi:10.1016/S0028-3932(99)00146-3
Raaphorst J, de Visser M, Linssen WH, de Haan RJ, Schmand B (2010) The cognitive profile of amyotrophic lateral sclerosis: a meta-analysis. Amyotroph Lateral Scler 11:27–37. doi:10.3109/17482960802645008
Taylor LJ, Brown RG, Tsermentseli S, Al-Chalabi A, Shaw CE, Ellis CM, Leigh PN, Goldstein LH (2013) Is language impairment more common than executive dysfunction in amyotrophic lateral sclerosis? J Neurol Neurosurg Psychiatry 84:494–498
Abrahams S (2012) Social cognition in amyotrophic lateral sclerosis. Neurodegener Dis Manag 1:397–405
Cavallo M, Adenzato M, Macpherson SE, Karwig G, Enrici I, Abrahams S (2011) Evidence of social understanding impairment in patients with amyotrophic lateral sclerosis. PLoS One 6:e25948
Girardi A, Macpherson SE, Abrahams S (2011) Deficits in emotional and social cognition in amyotrophic lateral sclerosis. Neuropsychology 25:53–65
Abrahams S, Newton J, Niven E, Foley J, Bak TH (2014) Screening for cognition and behaviour changes in ALS. Amyotroph Lateral Scler Frontotemporal Degener 15:9–14
Bak TH, Hodges JR (2001) Motor neurone disease, dementia and aphasia: coincidence, co occurrence or continuum? J Neurol 248:260–270
Niven E, Newton J, Foley J, Colville S, Swingler R, Chandran S, Bak TH, Abrahams S (2015) Validation of the Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS): a cognitive tool for motor disorders. Amyotroph Lateral Scler Frontotemporal Degener 16:172–179
Rascovsky K, Hodges JR, Knopman D, Mendez MF, Kramer JH, Neuhaus J et al (2011) Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 134:2456–2477
Snowden JS, Rollinson S, Thompson JC, Harris JM, Stopford CL, Richardson AM, Jones M, Gerhard A, Davidson YS, Robinson A, Gibbons L, Hu Q, DuPlessis D, Neary D, Mann DM, Pickering-Brown SM (2012) Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations. Brain 135:693–708
Lulé D, Burkhardt C, Abdulla S, Böhm S, Kollewe K, Uttner I, Abrahams S, Bak TH, Petri S, Weber M, Ludolph AC (2015) The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: a cross-sectional comparison of established screening tools in a German–Swiss population. Amyotroph Lateral Scler Frontotemporal Degener 16:16–23
Poletti B, Solca M, 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 A, Silani V (2016) The validation of the Italian Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Amyotroph Lateral Scler Frontotemporal Degener 24:1–10
Spinnler H, Tognoni G (1987) Standardizzazione e taratura italiana di test neuropsicologici. Ital J Neurol Sci 6(Suppl 8):8–20
Santangelo G, Siciliano M, Pedone R, Vitale C, Falco F, Bisogno R, Siano P, Barone P, Grossi D, Santangelo F, Trojano L (2015) Normative data for the Montreal Cognitive Assessment in an Italian population sample. Neurol Sci 36:585–591. doi:10.1007/s10072-014-1995-y
Wilks SS (1941) Determination of samples size for setting tolerance limits. Ann Math Stat 12:91–96
Ackermann H (1985) Mehrdimensionale nicht-parametrische Normbereiche. Methodologische und medizinische Aspekte. Springer, Berlin
Cohen JW (1988) Statistical power analysis for the behavioral sciences, 2nd edn. Lawrence Erlbaum Associates Publishers, Hillsdale
Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE (2005) Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology 65:586–590
Strong MJ, Grace GM, Freedman M, Lomen-Hoerth C, Woolley S, Goldstein LH, Murphy J, Shoesmith C, Rosenfeld J, Leigh PN, Bruijn L, Ince P, Figlewicz D (2009) Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler 10:131–146
Ardila A, Bertolucci PH, Braga LW, Castro-Caldas A, Judd T, Kosmidis MH, Matute E, Nitrini R, Ostrosky-Solis F, Rosselli M (2010) Illiteracy: the neuropsychology of cognition without reading. Arch Clin Neuropsychol 25:689–712
Ash S, Olm C, McMillan CT, Boller A, Irwin DJ, McCluskey L, Elman L, Grossman M (2015) Deficits in sentence expression in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 16:31–39. doi:10.3109/21678421.2014.974617
Trojano L, Siciliano M, Pedone R, Cristinzio C, Grossi D (2015) Italian normative data for the Battery for Visuospatial Abilities (TERADIC). Neurol Sci 36:1353–1361. doi:10.1007/s10072-015-2114-4
Burton LA, Henninger D, Hafetz J (2005) Gender differences in relations of mental rotation, verbal fluency, and SAT scores to finger length ratios as hormonal indexes. Dev Neuropsychol 28:493–505
Ganley CM, Vasilyeva M, Dulaney A (2014) Spatial ability mediates the gender difference in middle school students’ science performance. Child Dev 85:1419–1432. doi:10.1111/cdev.12230
Capitani E, Laiacona M (1997) Composite neuropsychological batteries and demographic correction: standardization based on equivalent scores, with a review of published data. The Italian Group for the Neuropsychological Study of Ageing. J Clin Exp Neuropsychol 19:795–809
Mioshi E, Dawson K, Mitchell J, Arnold R, Hodges JR (2006) The Addenbrooke’s Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening. Int J Geriatr Psychiatry 21:1078–1085. doi:10.1002/gps.1610
Siciliano M, Raimo S, Tufano D, Basile G, Grossi D, Santangelo F, Trojano L, Santangelo G (2016) The Addenbrooke’s Cognitive Examination Revised (ACE-R) and its sub-scores: normative values in an Italian population sample. Neurol Sci 37:385–392
Acknowledgements
The authors thank Dr. Barbara Poletti for her contribution in providing the Italian version of Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Moreover, the authors thank Dr. Marianna Vicidomini, Dr. Dalila Biondi, and Dr. Carla Caterino for their contribution in collecting data.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The authors have no conflict of interest to disclose.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Siciliano, M., Trojano, L., Trojsi, F. et al. Edinburgh Cognitive and Behavioural ALS Screen (ECAS)-Italian version: regression based norms and equivalent scores. Neurol Sci 38, 1059–1068 (2017). https://doi.org/10.1007/s10072-017-2919-4
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10072-017-2919-4