Measuring reliable change in cognition using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).

BACKGROUND: Cognitive impairment affects approximately 50% of people with amyotrophic lateral sclerosis (ALS). Research has indicated that impairment may worsen with disease progression. The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was designed to measure neuropsychological functioning in ALS, with its alternate forms (ECAS-A, B, and C) allowing for serial assessment over time. OBJECTIVE: The aim of the present study was to establish reliable change scores for the alternate forms of the ECAS, and to explore practice effects and test-retest reliability of the ECAS's alternate forms. METHOD: Eighty healthy participants were recruited, with 57 completing two and 51 completing three assessments. Participants were administered alternate versions of the ECAS serially (A-B-C) at four-month intervals. Intra-class correlation analysis was employed to explore test-retest reliability, while analysis of variance was used to examine the presence of practice effects. Reliable change indices (RCI) and regression-based methods were utilized to establish change scores for the ECAS alternate forms. RESULTS: Test-retest reliability was excellent for ALS Specific, ALS Non-Specific, and ECAS Total scores of the combined ECAS A, B, and C (all > .90). No significant practice effects were observed over the three testing sessions. RCI and regression-based methods produced similar change scores. CONCLUSION: The alternate forms of the ECAS possess excellent test-retest reliability in a healthy control sample, with no significant practice effects. The use of conservative RCI scores is recommended. Therefore, a change of ≥8, ≥4, and ≥9 for ALS Specific, ALS Non-Specific, and ECAS Total score is required for reliable change.

A Cross-sectional population-based investigation into behavioral change in amyotrophic lateral sclerosis: subphenotypes, staging, cognitive predictors, and survival.

OBJECTIVE: Amyotrophic Lateral Sclerosis (ALS) is a clinically heterogeneous neurodegenerative disorder associated with cognitive and behavioral impairment. The primary aim of this study was to identify behavioral subphenotypes in ALS using a custom designed behavioral assessment tool (Beaumont Behavioural Inventory, BBI). Secondary aims were to (1) investigate the predictive nature of cognitive assessment on behavioral change, (2) report the behavioral profile associated with the C9orf72 expansion, (3) categorize behavioral change through disease staging, and (4) to investigate the relationship between cross-sectional behavioral classification and survival. METHODS: A cross-sectional population-based research design was applied to examine behavioral data from ALS patients (n = 317) and healthy controls (n = 66). Patients were screened for the C9orf72 repeat expansion. A subcohort of ALS patients completed an extensive cognitive assessment battery (n = 65), to investigate predictors of behavior change. Principal component analysis (PCA) determined factors associated with altered behavior. Survival data were extracted from the Irish ALS register. RESULTS: No behavioral changes were reported in 180 patients (57%); 95 patients had mild-moderate behavioral change (30%); 42 patients met the cut-off for Clinically Severe Behavioral Change (13%), suggestive of a bvFTD diagnosis. The most frequently endorsed behaviors in ALS were reduced concern for hygiene (36.8%), irritability (36.2%), new unusual habits (33.4%), and increased apathy (31.1%). Five independent factors were identified through factor analysis. Social cognitive performance was predictive of behavior change (P = 0.031), yielding an R2 = 0.188. Behavioral categorization (mild/moderate/severe) at the time of assessment was not associated with survival (P = 0.198). INTERPRETATION: These data imply the presence of distinct subphenotypes of behavioral change in ALS, which most likely reflect subcategories of extramotor network disruption.

Identifying behavioural changes in ALS: Validation of the Beaumont Behavioural Inventory (BBI).

OBJECTIVE: Behavioural changes are an important part of amyotrophic lateral sclerosis (ALS). However, most tools do not account for the influence of motor impairment. Furthermore, they do not fully measure the broad range of behavioural changes specific to ALS. This study aimed to develop and validate an ALS specific behavioural inventory, the Beaumont Behavioural Inventory (BBI). METHODS: The BBI was validated in a cohort of ALS patients (n = 85) and 78 age-, gender-, and education-matched controls. The scale was validated against the Frontal Systems Behaviour Scale (FrSBe) and The Frontal Assessment Battery (FAB) for convergent validity, and against other non-behavioural measures to assess discriminant validity. Reliability was assessed with Cronbach's alpha. RESULTS: The instrument showed high internal consistency (Cronbach's alpha value =0.891). BBI scores highly correlated with the FrSBe and moderately with the FAB. However, the measure was independent from non-behavioural measures. Using a cut-off score of 7 for mild behavioural changes, the BBI displayed high sensitivity and specificity (87.9% and 78.85%, respectively). The cut-off score for moderate changes, consistent with a diagnosis of ALS-FTD, is set at 22.5, showing 90% sensitivity and 96% specificity. DISCUSSION: The BBI is a sensitive and specific tool to assess the entire behavioural spectrum of ALS.