{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T22:20:07Z","timestamp":1792189207342,"version":"4.4.0"},"reference-count":25,"publisher":"BMJ","issue":"7","funder":[{"name":"Joint Programme - Neurodegenerative Disease Research","award":["Brain-Mend"],"award-info":[{"award-number":["Brain-Mend"]}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF-2010-2309849"],"award-info":[{"award-number":["RF-2010-2309849"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF-2011-02351193"],"award-info":[{"award-number":["RF-2011-02351193"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100011102","name":"Seventh Framework Programme","doi-asserted-by":"publisher","award":["259867"],"award-info":[{"award-number":["259867"]}],"id":[{"id":"10.13039\/100011102","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"accepted":{"date-parts":[[2019,1,18]]},"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>In 2017, the diagnostic criteria for cognitive and behavioural impairment in amyotrophic lateral sclerosis (ALS) with frontotemporal dementia (ALSFTD-1) have been modified (ALSFTD-2) with the inclusion of a novel category (ALS with combined cognitive and behavioural impairment, ALScbi) and with changes of operational criteria of the other categories (ALS with cognitive impairment (ALSci), ALS with behavioural impairment (ALSbi) and ALS with frontotemporal dementia (ALS-FTD)). We compared the two sets of criteria to assess the effect of the revised criteria on the cognitive classification of patients with ALS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Two cohorts of patients with ALS were included in this study: a population-based cohort including patients identified through the Piemonte\/Valle d\u2019Aosta register for ALS in the 2014\u20132017 period (n=321), and a referral cohort recruited at the Turin ALS centre and at the ALS centre of the Maugeri Institute in Milan in the same period (n=205). Cognitive function was classified in blind by two neuropsychologists expert in ALS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>ALSFTD-2 criteria determined a shift of about 15% of patients from their original category to a new one. In both cohorts, about 9% of patients were reclassified to the novel category ALScbi. Among patients previously classified as cognitively normal, 14 (4.3%, population-based cohort) and 19 (9.3%, referral cohort) were reclassified as ALSbi or ALSci. The median survival of the different categories was significantly different with both with sets of criteria.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The new ALSFTD-2 criteria, compared with the old ones, have positive effects on the clinical practice being more sensitive to the early cognitive impairment and having a better prognostic yield.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jnnp-2018-319696","type":"journal-article","created":{"date-parts":[[2019,2,8]],"date-time":"2019-02-08T10:40:59Z","timestamp":1549622459000},"page":"734-739","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":25,"title":["Validation of the revised classification of cognitive and behavioural impairment in ALS"],"prefix":"10.1136","volume":"90","author":[{"given":"Barbara","family":"Iazzolino","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Debora","family":"Pain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Peotta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5122-7243","authenticated-orcid":false,"given":"Andrea","family":"Calvo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Moglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Canosa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Umberto","family":"Manera","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Ilardi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Bombaci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Pierre","family":"Zucchetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriele","family":"Mora","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9579-5341","authenticated-orcid":false,"given":"Adriano","family":"Chio","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2019,2,7]]},"reference":[{"key":"2024120416214240000_90.7.734.1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-300188"},{"key":"2024120416214240000_90.7.734.2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2013-307223"},{"key":"2024120416214240000_90.7.734.3","doi-asserted-by":"publisher","DOI":"10.1080\/17482960802654364"},{"key":"2024120416214240000_90.7.734.4","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1080\/21678421.2016.1267768","article-title":"Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria","volume":"18","author":"Strong","year":"2017","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2024120416214240000_90.7.734.5","doi-asserted-by":"crossref","first-page":"1097","DOI":"10.1001\/jamaneurol.2017.1387","article-title":"Secular trends of amyotrophic lateral sclerosis: the Piemonte and Valle d'Aosta register","volume":"74","author":"Chi\u00f2","year":"2017","journal-title":"JAMA Neurol"},{"key":"2024120416214240000_90.7.734.6","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awr179"},{"key":"2024120416214240000_90.7.734.7","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1080\/21678421.2016.1183679","article-title":"The validation of the Italian Edinburgh cognitive and behavioural ALS screen (ECAS)","volume":"17","author":"Poletti","year":"2016","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2024120416214240000_90.7.734.8","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.51.6.1546"},{"key":"2024120416214240000_90.7.734.9","unstructured":"Grace J , Malloy P . Frontal systems behavior scale (FrSBe): professional manual. Lutz, Fla: Psychological Assessment Resources, 2001."},{"key":"2024120416214240000_90.7.734.10","doi-asserted-by":"crossref","unstructured":"Gibbons CJ , Mills RJ , Thornton EW , et al . Rasch analysis of the hospital anxiety and depression scale (hads) for use in motor neurone disease. Health Qual Life Outcomes 2011;9.doi:10.1186\/1477-7525-9-82","DOI":"10.1186\/1477-7525-9-82"},{"key":"2024120416214240000_90.7.734.11","doi-asserted-by":"publisher","DOI":"10.1093\/neucas\/7.1.31"},{"key":"2024120416214240000_90.7.734.12","doi-asserted-by":"publisher","DOI":"10.1177\/001316446002000104"},{"key":"2024120416214240000_90.7.734.13","doi-asserted-by":"publisher","DOI":"10.2307\/2529310"},{"key":"2024120416214240000_90.7.734.14","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1155\/2013\/126010","article-title":"The cognitive and behavioural profile of amyotrophic lateral sclerosis: application of the consensus criteria","volume":"27","author":"Consonni","year":"2013","journal-title":"Behav Neurol"},{"key":"2024120416214240000_90.7.734.15","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0087163","article-title":"Spectrum of cognitive impairment in Korean ALS patients without known genetic mutations","volume":"9","author":"Oh","year":"2014","journal-title":"PLoS One"},{"key":"2024120416214240000_90.7.734.16","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000106827.39764.7E"},{"key":"2024120416214240000_90.7.734.17","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181f5d4c4"},{"key":"2024120416214240000_90.7.734.18","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.62.5.464"},{"key":"2024120416214240000_90.7.734.19","doi-asserted-by":"crossref","first-page":"e1370","DOI":"10.1212\/WNL.0000000000006317","article-title":"ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS","volume":"91","author":"Crockford","year":"2018","journal-title":"Neurology"},{"key":"2024120416214240000_90.7.734.20","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/j.cortex.2016.04.014","article-title":"Beyond the consensus criteria: multiple cognitive profiles in amyotrophic lateral sclerosis?","volume":"81","author":"Consonni","year":"2016","journal-title":"Cortex"},{"key":"2024120416214240000_90.7.734.21","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1212\/01.wnl.0000281904.21495.cb","article-title":"Effect of referral bias on assessing survival in ALS","volume":"69","author":"Logroscino","year":"2007","journal-title":"Neurology"},{"key":"2024120416214240000_90.7.734.22","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0195821","article-title":"Referral bias in ALS epidemiological studies","volume":"13","author":"Logroscino","year":"2018","journal-title":"PLoS One"},{"key":"2024120416214240000_90.7.734.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.eurpsy.2008.09.001"},{"key":"2024120416214240000_90.7.734.24","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2017-316277"},{"key":"2024120416214240000_90.7.734.25","doi-asserted-by":"crossref","unstructured":"Lul\u00e9 DE , Aho-\u00d6zhan HEA , V\u00e1zquez C , et al . Story of the ALS-FTD continuum retold: rather two distinct entities. J Neurol Neurosurg Psychiatry 2018:jnnp-2018-318800.doi:10.1136\/jnnp-2018-318800","DOI":"10.1136\/jnnp-2018-318800"}],"container-title":["Journal of Neurology, Neurosurgery &amp; Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2018-319696","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,5]],"date-time":"2024-12-05T00:23:27Z","timestamp":1733358207000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2018-319696"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2,7]]},"references-count":25,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2019,6,13]]},"published-print":{"date-parts":[[2019,7]]}},"alternative-id":["10.1136\/jnnp-2018-319696"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2018-319696","relation":{},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,2,7]]}}}