Assessing behaviour in ALS: the importance of using disease-specific tools

author: Marta Pinto-Grau, Trinity Biomedical Sciences Institute, Trinity College Dublin
published: July 21, 2017,   recorded: May 2017,   views: 854


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Background: Behavioural changes are a core aspect of Amyotrophic Lateral Sclerosis (ALS). General behavioural assessments, such as the Frontal Systems Behaviour Scale (FrSBe), have been widely used to assess behaviour in ALS, although these measures tend to overestimate the presence of changes as the influence of motor dysfunction on behaviour is not considered. ALS-specific behavioural scales correcting for such confounders have been developed. The Beaumont Behavioural Inventory (BBI) is a 41-item proxy-report questionnaire which assesses the whole spectrum of behaviours observed in ALS. The BBI has shown high internal consistency (Cronbach’s alpha=0.891). Objective: This work aims to compare two BBI validation studies, one using the FrSBe as the gold standard, and the other comparing it against another ALS-specific tool, the ALS-FTD-Q. Methods: The BBI has been validated against the FrSBe in a sample of 85 ALS patients, and has also been compared to the ALS-FTD-Q in an additional sample of 60 patients. Results: In both studies, the BBI demonstrated good construct validity. Highly significant positive correlations were observed between the BBI and the FrSBe (r=0.760, p<.0001), and the BBI and the ALS-FTD-Q (r=.807, p<.0001), indicating adequate convergent validity. In both studies, no correlations were observed between the BBI and non-behavioural measures, which indicated good discriminant validity. A cut-off score of ≥7 for the presence of behavioural changes was derived from an age-, gender-, and education-matched healthy control sample (n=78). When validated against the FrSBe, this cut-off showed high sensitivity (88%) and specificity (79%). When compared to the ALS-FTD-Q, high sensitivity was observed (100%), but specificity was decreased (70%). Further analysis of behavioural aspects endorsed by cases classified as ‘false positives’ showed that changes most frequently reported included behavioural aspects not measured by the ALS-FTD-Q, such as diminished social interest, excessive sensitivity to sensory stimuli, and the presence of grammatical mistakes. Conclusions: General behavioural instruments that do not correct for motor disability tend to overestimate the presence of behavioural changes in ALS. Disease-specific instruments that do not include the whole range of behaviours characteristic of ALS tend to underestimate its presence. The BBI overcomes both limitations and should be considered the gold standard to assess behaviour in ALS.

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