RESUMO
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition with observable cognitive and behavioral impairment. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a tool developed specifically for people with ALS (pwALS) and previously translated into Russian, but the psychometric properties have not yet been explored. The aim was to explore and determine the psychometric properties of the Russian-version of ECAS (ECAS-R). METHODS: 56 Russian speaking pwALS, 32 of their caregivers and 26 healthy controls were recruited for the study. They completed the ECAS-R, Patient Health Questionnaire-9 (PHQ-9) and Montreal Cognitive Assessment (MoCA). King Staging System was also utilized. Internal consistency, divergent and convergent validity, as well as culturally-derived cutoff scores of ECAS-R were determined. RESULTS: The internal consistency of ECAS-R was good (Cronbach's alpha = 0.73). Convergent validity was observed though a strong correlation between the ECAS-R and MoCA scores. No correlation between ECAS-R and PHQ-9 were observed in terms of divergent validity. Based on culturally-derived cutoff scores, 64.2% (N = 36) of pwALS displayed cognitive impairment, with the most affected cognitive domains as executive function and language. Apathy was the most common behavioral impairment for pwALS followed by a loss of sympathy/empathy. CONCLUSIONS: The ECAS-R is valid and reliable tool for the screening for the cognitive and behavioral impairment in Russian-speaking pwALS, with culturally-derived cutoffs presented.
RESUMO
Objectives: To determine the frequency and correlates of apathy in amyotrophic lateral sclerosis (ALS) and its influence on the prognosis of the disease.Methods: Three databases were searched: MEDLINE, PubMed, and Google Scholar. Quantitative synthesis of the frequency of apathy in ALS was conducted using random effects in Stata (College Station, TX). Meta-regression and subgroup analyses were conducted to investigate the association between frequency of apathy in ALS and different covariates.Results: Fifty-two studies (51 cohorts) were included in the analysis. The pooled frequency of apathy in ALS was 25% (95% confidence interval (CI) 14-35%) according to the studies that used self-rated tools and 34% (95% CI 27-41%) according to studies that used informant-rated tools. The emergence of apathy was associated with cognitive decline and bulbar onset of the disease. There was no consistent relationship between apathy and disease stage or the severity of depression. Structural brain imaging studies established that ALS patients with apathy exhibited more prominent changes with structural and functional brain imaging particularly involving fronto-subcortical regions of the brain. Overall, apathy worsened the long-term prognosis of ALS.Discussion: Apathy affects up to a third of ALS patients аnd develops in the context of progressive neurodegeneration. Increased awareness and understanding of non-motor symptoms in ALS highlights the potential utility of apathy as an outcome measure in future clinical trial design.
