{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T12:35:44Z","timestamp":1780403744958,"version":"3.54.1"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2024,4,14]],"date-time":"2024-04-14T00:00:00Z","timestamp":1713052800000},"content-version":"vor","delay-in-days":13,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["EXC 2145 SyNergy \u2013 ID 390857198"],"award-info":[{"award-number":["EXC 2145 SyNergy \u2013 ID 390857198"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004587","name":"Instituto de Salud Carlos III","doi-asserted-by":"publisher","award":["PI20\/00448"],"award-info":[{"award-number":["PI20\/00448"]}],"id":[{"id":"10.13039\/501100004587","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100015873","name":"National Brain Appeal","doi-asserted-by":"publisher","award":["RCN 290173"],"award-info":[{"award-number":["RCN 290173"]}],"id":[{"id":"10.13039\/100015873","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"publisher","award":["SM\u2010UCLO\u2010MA\u20100519"],"award-info":[{"award-number":["SM\u2010UCLO\u2010MA\u20100519"]}],"id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012317","name":"UCLH Biomedical Research Centre","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100012317","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["103838"],"award-info":[{"award-number":["103838"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["alz-journals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Alz &amp;amp; Dem Diag Ass &amp;amp; Dis Mo"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>INTRODUCTION<\/jats:title><jats:p>We aimed to expand the range of the frontotemporal dementia (FTD) phenotypes assessed by the Clinical Dementia Rating Dementia Staging Instrument plus National Alzheimer's Coordinating Center Behavior and Language Domains (CDR plus NACC FTLD).<\/jats:p><\/jats:sec><jats:sec><jats:title>METHODS<\/jats:title><jats:p>Neuropsychiatric and motor domains were added to the standard CDR plus NACC FTLD generating a new CDR plus NACC FTLD\u2010NM scale. This was assessed in 522 mutation carriers and 310 mutation\u2010negative controls from the Genetic Frontotemporal dementia Initiative (GENFI).<\/jats:p><\/jats:sec><jats:sec><jats:title>RESULTS<\/jats:title><jats:p>The new scale led to higher global severity scores than the CDR plus NACC FTLD: 1.4% of participants were now considered prodromal rather than asymptomatic, while 1.3% were now considered symptomatic rather than asymptomatic or prodromal. No participants with a clinical diagnosis of an FTD spectrum disorder were classified as asymptomatic using the new scales.<\/jats:p><\/jats:sec><jats:sec><jats:title>DISCUSSION<\/jats:title><jats:p>Adding new domains to the CDR plus NACC FTLD leads to a scale that encompasses the wider phenotypic spectrum of FTD with further work needed to validate its use more widely.<\/jats:p><\/jats:sec><jats:sec><jats:title>Highlights<\/jats:title><jats:p><jats:list list-type=\"bullet\">\n<jats:list-item><jats:p>The new Clinical Dementia Rating Dementia Staging Instrument plus National Alzheimer's Coordinating Center Behavior and Language Domains neuropsychiatric and motor (CDR plus NACC FTLD\u2010NM) rating scale was significantly positively correlated with the original CDR plus NACC FTLD and negatively correlated with the FTD Rating Scale (FRS).<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>No participants with a clinical diagnosis in the frontotemporal dementia spectrum were classified as asymptomatic with the new CDR plus NACC FTLD\u2010NM rating scale.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>Individuals had higher global severity scores with the addition of the neuropsychiatric and motor domains.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>A receiver operating characteristic analysis of symptomatic diagnosis showed nominally higher areas under the curve for the new scales.<\/jats:p><\/jats:list-item>\n<\/jats:list><\/jats:p><\/jats:sec>","DOI":"10.1002\/dad2.12571","type":"journal-article","created":{"date-parts":[[2024,4,15]],"date-time":"2024-04-15T01:55:55Z","timestamp":1713146155000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Extending the phenotypic spectrum assessed by the CDR plus NACC FTLD in genetic frontotemporal dementia"],"prefix":"10.1002","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3105-7099","authenticated-orcid":false,"given":"Kiran","family":"Samra","sequence":"first","affiliation":[{"name":"Dementia Research Centre Department of Neurodegenerative Disease UCL Queen Square Institute of Neurology  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georgia","family":"Peakman","sequence":"additional","affiliation":[{"name":"Dementia Research Centre Department of Neurodegenerative Disease UCL Queen Square Institute of Neurology  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amy M.","family":"MacDougall","sequence":"additional","affiliation":[{"name":"Department of Medical Statistics London School of Hygiene and Tropical Medicine  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arabella","family":"Bouzigues","sequence":"additional","affiliation":[{"name":"Dementia Research Centre Department of 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