{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,25]],"date-time":"2026-10-25T16:27:32Z","timestamp":1792945652295,"version":"4.5.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T00:00:00Z","timestamp":1788998400000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Cognitive and behavioral dysfunction in frontotemporal dementia (FTD) evolves along a clinical continuum from a clinically silent stage of disease, through a prodromal period, often referred to as mild cognitive impairment (MCI) and\/or mild behavioral impairment (MBI), and into a clinically manifest stage recognized by the syndromes of behavioral variant FTD and primary progressive aphasia (PPA). Similar cognitive and behavioral manifestations of varying severity are also encountered in individuals with amyotrophic lateral sclerosis (ALS).<\/jats:p>\n                  <jats:p>Notwithstanding this phenotypic overlap as well as a degree of shared genetic risk and underlying pathology, the approach to phenotypic characterization and description meaningfully differs between the ALS and FTD communities. The problem is particularly evident within the ALS community when describing impairment in the prodromal vs. clinically manifest disease stages. Pertinent considerations include the nature of cognitive and behavioral assessments, the nosology used to describe symptoms identified including those encompassed by the terms \u2018behavioral\u2019 and \u2018neuropsychiatric\u2019, the operationalized criteria that define prodromal syndromes and clinically manifest disease, and the extent to which these criteria rely on longitudinal (vs. cross-sectional) data.<\/jats:p>\n                  <jats:p>A multi-stakeholder workshop, held in London, Ontario (May 12-14, 2025), brought together neurologists, psychiatrists, neuropsychologists, neuropathologists, neuroscientists, and neurogeneticists studying ALS and FTD, to promote inter-disciplinary dialogue and to define a research agenda that might be jointly tackled to address these critical issues. A new nosology for classifying frontotemporal-related behavioral and neuropsychiatric dysfunction, which could be used to support empirical evidence collection, is proposed alongside a list of cognitive, language, behavioral and neuropsychiatric symptoms to be considered in the evaluation of individuals with ALS. Research priorities for each of the ALS and FTD communities, as well as initiatives that should be undertaken jointly, are highlighted. The long-term goal is to harmonize approaches to phenotypic characterization, the nosology used to describe symptoms, and the criteria used to define landmark stages along the clinical continua of the motor neuron and frontotemporal axes irrespective of clinical stage.<\/jats:p>","DOI":"10.1093\/brain\/awag300","type":"journal-article","created":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T11:48:29Z","timestamp":1788954509000},"source":"Crossref","is-referenced-by-count":2,"title":["Conceptualizing cognition and behaviour across amyotrophic lateral sclerosis and frontotemporal dementia"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4241-5135","authenticated-orcid":false,"given":"Michael","family":"Benatar","sequence":"first","affiliation":[{"name":"Department of Neurology and the ALS Center, Miller School of Medicine, University of Miami , Miami, FL, 33129 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward D","family":"Huey","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Human Behavior, Alpert Medical School of Brown University , Providence, RI, 02906 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiadi U","family":"Onyike","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine , Baltimore, MD, 21287 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0103-8580","authenticated-orcid":false,"given":"Caroline","family":"McHutchison","sequence":"additional","affiliation":[{"name":"Division of Psychology, Faculty of Natural Sciences, University of Stirling , FK9 4LA ,","place":["UK"]},{"name":"Euan MacDonald Centre for MND Research, University of Edinburgh , EH16 4SB ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Carmela","family":"Tartaglia","sequence":"additional","affiliation":[{"name":"Division of Neurology University of Toronto , Toronto, ON, M5T 1A8 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan S","family":"Barker","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Human Behavior, Alpert Medical School of Brown University , Providence, RI, 02906 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Abrahams","sequence":"additional","affiliation":[{"name":"Euan MacDonald Centre for MND Research, University of Edinburgh , EH16 4SB ,","place":["UK"]},{"name":"Department of Psychology, School of Philosophy, Psychology, and Language Sciences, University of Edinburgh , EH8 9JZ ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joke","family":"De Vocht","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Experimental Neurology, Leuven Brain Institute , KU Leuven, Leuven, 3000 ,","place":["Belgium"]},{"name":"Department of Neurology, University Hospitals Leuven , Leuven, 3000 ,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9053-0114","authenticated-orcid":false,"given":"Emma","family":"Rhodes","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania Perelman School of Medicine , Philadelphia, PA, 19104 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yolande A","family":"Pijnenburg","sequence":"additional","affiliation":[{"name":"Department of Neurology, Alzheimer Center Amsterdam, Amsterdam UMC, Amsterdam Neuroscience , Amsterdam, 1081HZ ,","place":["The Netherlands"]},{"name":"Amsterdam Neuroscience, Neurodegeneration , Amsterdam, 1081HZ ,","place":["The Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0267-3180","authenticated-orcid":false,"given":"Martin R","family":"Turner","sequence":"additional","affiliation":[{"name":"Nuffield Department of Clinical Neurosciences, University of Oxford , Oxford, OX3 9DU ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4010-2357","authenticated-orcid":false,"given":"Philip","family":"van Damme","sequence":"additional","affiliation":[{"name":"Department of Neurosciences, Experimental Neurology, Leuven Brain Institute , KU Leuven, Leuven, 3000 ,","place":["Belgium"]},{"name":"Department of Neurology, University Hospitals Leuven , Leuven, 3000 ,","place":["Belgium"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4589-1180","authenticated-orcid":false,"given":"Edward B","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania , Philadelphia, PA, 19104 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne","family":"Wuu","sequence":"additional","affiliation":[{"name":"Department of Neurology and the ALS Center, Miller School of Medicine, University of Miami , Miami, FL, 33129 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8703-6940","authenticated-orcid":false,"given":"Alberto","family":"Benussi","sequence":"additional","affiliation":[{"name":"Neurology Unit, Department of Medical, Surgical and Health Sciences, University of Trieste , Trieste, 34149 ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4153-8187","authenticated-orcid":false,"given":"Bradley F","family":"Boeve","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mayo Clinic , Rochester, MN, 55905 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corey T","family":"McMillan","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Pennsylvania Perelman School of Medicine , Philadelphia, PA, 19104 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J","family":"Strong","sequence":"additional","affiliation":[{"name":"Molecular Medicine Group, Robarts Research Institute, Schulich School of Medicine & Dentistry, Western University , London, N6A 3K7 ,","place":["Canada"]},{"name":"Department of Clinical Neurological Sciences, Schulich School of Medicine & Dentistry, Western University , London, N6A 3K7 ,","place":["Canada"]},{"name":"Department of Pathology and Laboratory Medicine, Schulich School of Medicine & Dentistry, Western University , London, N6A 3K7 ,","place":["Canada"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,9,10]]},"container-title":["Brain"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/brain\/advance-article-pdf\/doi\/10.1093\/brain\/awag300\/71018054\/awag300.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/brain\/advance-article-pdf\/doi\/10.1093\/brain\/awag300\/71018054\/awag300.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T10:23:29Z","timestamp":1789035809000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/brain\/advance-article\/doi\/10.1093\/brain\/awag300\/8789855"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,10]]},"references-count":0,"URL":"https:\/\/doi.org\/10.1093\/brain\/awag300","relation":{},"ISSN":["0006-8950","1460-2156"],"issn-type":[{"value":"0006-8950","type":"print"},{"value":"1460-2156","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,10]]},"article-number":"awag300"}}