Prioritizing neuropsychological research and care in Amyotrophic Lateral Sclerosis (ALS): building an international neuropsychological framework for ALS

Abstract

Background Neuropsychological changes, long overlooked in Amyotrophic lateral Sclerosis (ALS), are now recognized as an integral part of its clinical spectrum, impacting survival, patient care and clinical trial eligibility (Citation1). Up to 50% of people with ALS (PwALS) can exhibit neuropsychological changes, as seen in Frontotemporal Dementia, with up to 15% of PwALS meeting diagnostic criteria for concomitant FTD (Citation2). Cognitive symptoms include executive, verbal fluency, social cognition and language deficits. Behavioral symptoms include disinhibition, apathy, diminished empathy, stereotyped or perseverative behaviors, and hyperorality. Anosognosia - impaired awareness of cognitive and behavioral deficits - is frequently observed, and psychotic symptoms may occur in a subset of PwALS (Citation2). Consequently, guidelines by the European Academy for Neurology on the management of ALS stress the importance of systemic neuropsychological assessments (Citation3). Despite these guidelines, neuropsychological assessment remains inconsistently applied across clinical and research settings and is underutilized in ALS clinical trials (Citation4). To address this critical issue, the International Consortium for Neuropsychology in ALS (ICNALS) was founded, serving as a dedicated platform for clinicians and researchers addressing neuropsychological aspects of ALS. Its primary objective is to foster collaboration, best-practice sharing, and to elevate standards of ALS clinical care and research. This commentary outlines the formation of ICNALS and provides an overview of its first workshop in Turin, highlighting emerging priorities for the field, identified by attendees.