{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T09:25:31Z","timestamp":1759483531314},"reference-count":19,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2012,8,13]],"date-time":"2012-08-13T00:00:00Z","timestamp":1344816000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"},{"start":{"date-parts":[[2012,8,13]],"date-time":"2012-08-13T00:00:00Z","timestamp":1344816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/2.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Alz Res Therapy"],"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                        <jats:title>Introduction<\/jats:title>\n                        <jats:p>An expanded hexanucleotide repeat in the <jats:italic>C9ORF72<\/jats:italic> gene has recently been identified as an important cause of frontotemporal dementia and motor neuron disease; however, the phenotypic spectrum of this entity and its pathophysiologic basis have yet to be fully defined. Psychiatric features may be early and prominent, although a putative cortico-thalamo-cerebellar network has been implicated in the pathogenesis of the clinical phenotype. Differentiation of self from others is a core cognitive operation that could potentially link network disintegration with neuropsychiatric symptoms in <jats:italic>C9ORF72<\/jats:italic>-associated frontotemporal dementia.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Methods<\/jats:title>\n                        <jats:p>We undertook a detailed behavioral analysis of self-other attribution in a 67-year-old male patient with behavioral variant frontotemporal dementia (bvFTD) due to the <jats:italic>C9ORF72<\/jats:italic> expansion by using a novel paradigm requiring differentiation of the effects of self- and non-self-generated actions. The patient's performance was assessed in relation to two older male patients with bvFTD not attributable to the <jats:italic>C9ORF72<\/jats:italic> expansion and four healthy older male subjects.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Results<\/jats:title>\n                        <jats:p>Compared with the healthy control group, the patient with the <jats:italic>C9OFR72<\/jats:italic> mutation showed a deficit of self-other differentiation that was disproportionate to his otherwise relatively indolent clinical phenotype. The performance of the other patients with bvFTD was similar to that of healthy subjects.<\/jats:p>\n                     <\/jats:sec><jats:sec>\n                        <jats:title>Conclusion<\/jats:title>\n                        <jats:p>We propose that impaired self-other differentiation is a candidate mechanism for neuropsychiatric decline in association with the <jats:italic>C9ORF72<\/jats:italic> expansion. We offer this preliminary observation as a stimulus to further work.<\/jats:p>\n                     <\/jats:sec>","DOI":"10.1186\/alzrt145","type":"journal-article","created":{"date-parts":[[2012,9,28]],"date-time":"2012-09-28T02:00:30Z","timestamp":1348797630000},"update-policy":"http:\/\/dx.doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Impaired self-other differentiation in frontotemporal dementia due to the C9ORF72 expansion"],"prefix":"10.1186","volume":"4","author":[{"given":"Laura E","family":"Downey","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Colin J","family":"Mahoney","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin N","family":"Rossor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian J","family":"Crutch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jason D","family":"Warren","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2012,8,13]]},"reference":[{"key":"123_CR1","doi-asserted-by":"publisher","first-page":"736","DOI":"10.1093\/brain\/awr361","volume":"135","author":"CJ Mahoney","year":"2012","unstructured":"Mahoney CJ, Beck J, Rohrer JD, Lashley T, Mok K, Shakespeare T, Yeatman T, Warrington EK, Schott JM, Fox NC, Hardy J, Colling J, Ravesz T, Mean S, Warren JD: Frontotemporal dementia with the C9ORF72 hexanucleotide repeat expansion: clinical, neuroanatomical and neuropathological features. 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