{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T13:33:08Z","timestamp":1790947988346,"version":"4.1.0"},"reference-count":94,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T00:00:00Z","timestamp":1655856000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NINDS R01NS050915"],"award-info":[{"award-number":["NINDS R01NS050915"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIDCD K24DC015544"],"award-info":[{"award-number":["NIDCD K24DC015544"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA P50AG023501"],"award-info":[{"award-number":["NIA P50AG023501"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA P01AG019724"],"award-info":[{"award-number":["NIA P01AG019724"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA R01AG038791"],"award-info":[{"award-number":["NIA R01AG038791"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NINDS U54NS092089"],"award-info":[{"award-number":["NINDS U54NS092089"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA K08AG052648"],"award-info":[{"award-number":["NIA K08AG052648"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA R01AG029577"],"award-info":[{"award-number":["NIA R01AG029577"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["NIA P50AG023501"],"award-info":[{"award-number":["NIA P50AG023501"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100017794","name":"Alzheimer\u2019s Disease Research Center of California","doi-asserted-by":"publisher","award":["03-75271 DHS\/ADP\/ARCC"],"award-info":[{"award-number":["03-75271 DHS\/ADP\/ARCC"]}],"id":[{"id":"10.13039\/100017794","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001167","name":"Larry L. Hillblom Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001167","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100019250","name":"John Douglas French Alzheimer\u2019s Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100019250","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100001212","name":"Koret Family Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001212","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Consortium for Frontotemporal Dementia Research"},{"name":"McBean Family Foundation"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,21]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Focal anterior temporal lobe degeneration often preferentially affects the left or right hemisphere. While patients with left-predominant anterior temporal lobe atrophy show severe anomia and verbal semantic deficits and meet criteria for semantic variant primary progressive aphasia and semantic dementia, patients with early right anterior temporal lobe atrophy are more difficult to diagnose as their symptoms are less well understood. Focal right anterior temporal lobe atrophy is associated with prominent emotional and behavioural changes, and patients often meet, or go on to meet, criteria for behavioural variant frontotemporal dementia. Uncertainty around early symptoms and absence of an overarching clinico-anatomical framework continue to hinder proper diagnosis and care of patients with right anterior temporal lobe disease. Here, we examine a large, well-characterized, longitudinal cohort of patients with right anterior temporal lobe-predominant degeneration and propose new criteria and nosology.<\/jats:p>\n                  <jats:p>We identified individuals from our database with a clinical diagnosis of behavioural variant frontotemporal dementia or semantic variant primary progressive aphasia and a structural MRI (n = 478). On the basis of neuroimaging criteria, we defined three patient groups: right anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n = 46), frontal-predominant atrophy with relative sparing of the right anterior temporal lobe (n = 79) and left-predominant anterior temporal lobe-predominant atrophy with relative sparing of the frontal lobes (n = 75). We compared the clinical, neuropsychological, genetic and pathological profiles of these groups.<\/jats:p>\n                  <jats:p>In the right anterior temporal lobe-predominant group, the earliest symptoms were loss of empathy (27%), person-specific semantic impairment (23%) and complex compulsions and rigid thought process (18%). On testing, this group exhibited greater impairments in Emotional Theory of Mind, recognition of famous people (from names and faces) and facial affect naming (despite preserved face perception) than the frontal- and left-predominant anterior temporal lobe-predominant groups. The clinical symptoms in the first 3 years of the disease alone were highly sensitive (81%) and specific (84%) differentiating right anterior temporal lobe-predominant from frontal-predominant groups. Frontotemporal lobar degeneration-transactive response DNA binding protein (84%) was the most common pathology of the right anterior temporal lobe-predominant group.<\/jats:p>\n                  <jats:p>Right anterior temporal lobe-predominant degeneration is characterized by early loss of empathy and person-specific knowledge, deficits that are caused by progressive decline in semantic memory for concepts of socioemotional relevance. Guided by our results, we outline new diagnostic criteria and propose the name, \u2018semantic behavioural variant frontotemporal dementia\u2019, which highlights the underlying cognitive mechanism and the predominant symptomatology. These diagnostic criteria will facilitate early identification and care of patients with early, focal right anterior temporal lobe degeneration as well as in vivo prediction of frontotemporal lobar degeneration-transactive response DNA binding protein pathology.<\/jats:p>","DOI":"10.1093\/brain\/awac217","type":"journal-article","created":{"date-parts":[[2022,6,22]],"date-time":"2022-06-22T09:59:28Z","timestamp":1655891968000},"page":"4080-4096","source":"Crossref","is-referenced-by-count":154,"title":["Right temporal degeneration and socioemotional semantics: semantic behavioural variant frontotemporal dementia"],"prefix":"10.1093","volume":"145","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9912-1616","authenticated-orcid":false,"given":"Kyan","family":"Younes","sequence":"first","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Department of Neurology and Neurological Sciences, Stanford University , Stanford, CA 94304 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Borghesani","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxime","family":"Montembeault","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3570-9143","authenticated-orcid":false,"given":"Salvatore","family":"Spina","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Luisa","family":"Mandelli","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariane E","family":"Welch","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Weis","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick","family":"Callahan","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanny M","family":"Elahi","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alice Y","family":"Hua","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David C","family":"Perry","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Karydas","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2896-3450","authenticated-orcid":false,"given":"Daniel","family":"Geschwind","sequence":"additional","affiliation":[{"name":"Neurogenetics Program, Department of Neurology and Semel Institute for Neuroscience and Human Behavior, David Geffen School of Medicine, University of California , Los Angeles, CA 90024 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5381-3801","authenticated-orcid":false,"given":"Eric","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of California , San Francisco, CA 94143 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6809-0618","authenticated-orcid":false,"given":"Lea T","family":"Grinberg","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Department of Pathology, University of California , San Francisco, CA 94143 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel H","family":"Kramer","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam L","family":"Boxer","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gil D","family":"Rabinovici","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Howard J","family":"Rosen","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William W","family":"Seeley","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Department of Pathology, University of California , San Francisco, CA 94143 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5991-3053","authenticated-orcid":false,"given":"Zachary A","family":"Miller","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruce L","family":"Miller","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Virginia E","family":"Sturm","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine P","family":"Rankin","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Luisa","family":"Gorno-Tempini","sequence":"additional","affiliation":[{"name":"Memory and Aging Center, Department of Neurology, UCSF Weill Institute for Neurosciences, University of California , San Francisco, CA 94158 , USA"},{"name":"Dyslexia Center, University of California , San Francisco, CA 94158 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,6,22]]},"reference":[{"key":"2023053018564389700_awac217-B1","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1136\/jnnp.57.4.416","article-title":"Clinical and neuropathological criteria for frontotemporal dementia","volume":"57","author":"Neary","year":"1994","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2023053018564389700_awac217-B2","first-page":"204","article-title":"Progressive right frontotemporal degeneration: Clinical, neuropsychological and SPECT characteristics","volume":"4","author":"Miller","year":"1993","journal-title":"Dementia"},{"key":"2023053018564389700_awac217-B3","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1212\/WNL.0b013e31821103e6","article-title":"Classification of primary progressive aphasia and its 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pathology","volume":"28","author":"Borghesani","year":"2020","journal-title":"NeuroImage Clin"},{"key":"2023053018564389700_awac217-B7","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1093\/brain\/awv387","article-title":"On the right side? A longitudinal study of left-versus right-lateralized semantic dementia","volume":"139","author":"Kumfor","year":"2016","journal-title":"Brain"},{"key":"2023053018564389700_awac217-B8","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1016\/j.nicl.2015.01.011","article-title":"Longitudinal gray matter contraction in three variants of primary progressive aphasia: A tenser-based morphometry study","volume":"8","author":"Brambati","year":"2015","journal-title":"NeuroImage Clin"},{"key":"2023053018564389700_awac217-B9","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41467-020-16089-9","article-title":"A unified neurocognitive model of semantics language social behaviour and face recognition in semantic dementia","volume":"11","author":"Ding","year":"2020","journal-title":"Nat Commun"},{"key":"2023053018564389700_awac217-B10","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1016\/j.cortex.2017.08.024","article-title":"Semantic dementia and the left and right 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