MOTOR NEURON DISEASE: Edited by Jinsy A. AndrewsCognitive and behavioural impairment in amyotrophic lateral sclerosisPender, Nialla,b; Pinto-Grau, Martaa,b; Hardiman, Orlaa,c Author Information aAcademic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College Dublin bDepartment of Psychology cDepartment of Neurology, Beaumont Hospital, Dublin, Ireland Correspondence to Orla Hardiman, BSc, MD, FRCPI, Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College Dublin, 152-160 Pearse Street, Dublin 2, Ireland. Tel: +353 18964496; e-mail: [email protected] Current Opinion in Neurology: October 2020 - Volume 33 - Issue 5 - p 649-654 doi: 10.1097/WCO.0000000000000862 Buy Metrics Abstract Purpose of review The current review provides an up to date overview of the nature and progression of the cognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS). Understanding these symptoms has implications for the management of the disease and the design of clinical trials, in addition to the support of patient and caregiver regarding mental capacity and end of life decision-making. Recent findings Cognitive and behavioural change in ALS are best characterized as the consequence of extensive network dysfunction. 35–45% of ALS patients present with mild–moderate cognitive impairment and comorbid dementia occurs in approximately 14% of patients, the majority of these meeting diagnostic criteria for frontotemporal dementia (FTD). Cognitive change in ALS manifests most commonly as executive dysfunction and language impairment. Behavioural change in the form of apathy, disinhibition, loss of sympathy and empathy, stereotyped behaviours and dietary changes occur. Summary Cognitive and behavioural impairment is an important feature of ALS, and reflects broad network dysfunction of frontostriatal and frontotemporal systems. Cognition and behaviour should be assessed early in the diagnostic process, and data driven approaches should be developed to enable reliable quantitative outcome assessment suitable for clinical trials. Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.