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Archives of Assessment Psychology

Abstract

Amyotrophic lateral sclerosis (ALS) is a multisystem neurodegenerative disorder in which cognitive, behavioral, language, and social-cognitive changes may occur across clinical phenotypes, including the ALS-frontotemporal spectrum. Neuropsychological assessment is important for characterizing nonmotor manifestations and identifying changes relevant to autonomy, everyday functioning, and clinical management. This systematic review examined literature identified through APA PsycINFO, PubMed, Google Scholar, and supplementary searching to characterize neuropsychological domains, measures, phenotype-related findings, and disease-specific limitations relevant to comprehensive assessment. Two hundred records underwent title and abstract screening, 150 reports were reviewed in full text, and 61 resources were retained for synthesis. ALS-specific screening instruments, including the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), support efficient identification of cognitive and behavioral abnormalities but provide less process-level detail than comprehensive neuropsychological assessment. Conventional measures also present validity challenges because dysarthria, limb weakness, fatigue, respiratory compromise, and loss of response capacity can affect performance in addition to the construct being assessed. Findings were synthesized into an evidence-informed, domain-standardized, measure-flexible framework covering language, verbal fluency, attention and working memory, learning and memory, visuospatial, visuoconstructional functioning, executive functioning, social cognition, behavioral functioning, apathy, mood and emotional functioning. The framework standardizes domain coverage while allowing assessment depth and measure selection to vary according to referral question, phenotype, disease stage, communication capacity, and physical burden. Prospective multicenter validation is needed to evaluate feasibility, reliability, validity, classification performance, responsiveness to change, and clinical utility.

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