{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T09:23:39Z","timestamp":1789982619211,"version":"4.0.1"},"reference-count":39,"publisher":"BMJ","issue":"2","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>Cognitive and behavioural changes within the spectrum of frontotemporal dementia (FTD) are observed frequently in patients with amyotrophic lateral sclerosis (ALS). Whether these changes also occur in other forms of motor neuron disease (MND) is not well studied. We therefore systemically screened a large cohort of patients with primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) for cognitive and behavioural changes, and subsequently compared our findings with a cohort of patients with ALS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Using a set of screening instruments (Edinburgh Cognitive\u00a0and Behavioural ALS Screen,  ALS and\u00a0Frontotemporal Dementia Questionnaire, Frontal Assessment\u00a0Battery,\u00a0and Hospital Anxiety and\u00a0Depression Scale), the presence of cognitive and behavioural changes as well as anxiety and depression in 277 patients with\u00a0ALS, 75 patients with\u00a0PLS and 143 patients with\u00a0PMA was evaluated retrospectively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found a high frequency of cognitive and behavioural abnormalities with similar profiles in all three groups. Subjects with behavioural variant FTD were identified in all groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The percentage of patients with\u00a0PLS and PMA with cognitive dysfunction was similar to patients with\u00a0ALS, emphasising the importance for cognitive screening as part of routine clinical care in all three patient groups. With a similar cognitive profile, in line with genetic and clinical overlap between the MNDs, the view of PLS as an MND exclusively affecting upper motor neurons and PMA exclusively affecting lower motor neurons cannot be held. Therefore, our findings are in contrast to the recently revised El Escorial criteria of 2015, where PLS and PMA are described as restricted phenotypes. Our study favours a view of PLS and PMA as multidomain diseases similar to ALS.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jnnp-2018-318788","type":"journal-article","created":{"date-parts":[[2018,8,3]],"date-time":"2018-08-03T16:15:28Z","timestamp":1533312928000},"page":"141-147","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":59,"title":["Cognitive and behavioural changes in PLS and PMA:challenging the concept of restricted phenotypes"],"prefix":"10.1136","volume":"90","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7277-1723","authenticated-orcid":false,"given":"B\u00e1lint S","family":"de Vries","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura M M","family":"Rustemeijer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonhard A","family":"Bakker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carin D","family":"Schr\u00f6der","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan H","family":"Veldink","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard H","family":"van den Berg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanja C W","family":"Nijboer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A","family":"van Es","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2018,8,3]]},"reference":[{"key":"2020050722330888000_90.2.141.1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2011-300188"},{"key":"2020050722330888000_90.2.141.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(16)00737-6"},{"key":"2020050722330888000_90.2.141.3","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1080\/21678421.2017.1354996","article-title":"A case series of PLS patients with frontotemporal dementia and overview of the literature","volume":"18","author":"de Vries","year":"2017","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2020050722330888000_90.2.141.4","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.204446"},{"key":"2020050722330888000_90.2.141.5","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1212\/WNL.0000000000000745","article-title":"Prefrontal involvement related to cognitive impairment in progressive muscular atrophy","volume":"83","author":"Raaphorst","year":"2014","journal-title":"Neurology"},{"key":"2020050722330888000_90.2.141.6","doi-asserted-by":"crossref","first-page":"291","DOI":"10.3109\/21678421.2015.1049183","article-title":"A revision of the El Escorial criteria - 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