{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T14:28:35Z","timestamp":1781188115081,"version":"3.54.1"},"reference-count":9,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clin Nucl Med"],"published-print":{"date-parts":[[2018,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n          <jats:p>A 57-year-old woman presented with a 3-month history of cognitive impairment, daytime somnolence, and violent sleep behavior. Her first- and second-degree relatives had similar symptoms prior to their premature deaths. Her MRI scan of the brain showed no significant abnormality. Electroencephalogram showed loss of slow-wave activity. Functional brain imaging performed with <jats:sup>18<\/jats:sup>F-FDG PET was fused with her MRI scans. This demonstrated profound hypometabolism in bilateral thalami and the posterior cingulate cortex, which is pathognomonic for familial fatal insomnia. Hypometabolism in the temporal lobes suggests a long-standing course of the disease. Genetic testing confirmed a mutation of the prion-protein gene (PRNP).<\/jats:p>","DOI":"10.1097\/rlu.0000000000002152","type":"journal-article","created":{"date-parts":[[2018,6,5]],"date-time":"2018-06-05T19:03:18Z","timestamp":1528225398000},"page":"e274-e275","source":"Crossref","is-referenced-by-count":6,"title":["18F-FDG PET Brain in a Patient With Fatal Familial Insomnia"],"prefix":"10.1097","volume":"43","author":[{"given":"Wei Ying","family":"Tham","sequence":"first","affiliation":[{"name":"Department of Nuclear Medicine and Molecular Imaging, Singapore General Hospital;"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yee-Liang","family":"Thian","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Imaging, National University Hospital; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pavanni","family":"Ratnagopal","sequence":"additional","affiliation":[{"name":"Neurology (SGH Campus), National Neuroscience Institute, Singapore."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wanying","family":"Xie","sequence":"additional","affiliation":[{"name":"Department of Nuclear Medicine and Molecular Imaging, Singapore General Hospital;"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"bib1-20230925","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1056\/NEJM198610163151605","article-title":"Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei","volume":"315","year":"1986","journal-title":"N Engl J Med"},{"key":"bib2-20230925","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1111\/j.1750-3639.1995.tb00576.x","article-title":"Fatal familial insomnia and familial Creutzfeldt-Jakob disease: clinical, pathological and molecular features","volume":"5","year":"1995","journal-title":"Brain Pathol"},{"key":"bib3-20230925","doi-asserted-by":"crossref","first-page":"774","DOI":"10.1136\/jnnp.68.6.774","article-title":"Fatal familial insomnia: clinical, neuropathological, and genetic description of a Spanish family","volume":"68","year":"2000","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"bib4-20230925","doi-asserted-by":"crossref","first-page":"444","DOI":"10.1056\/NEJM199202133260704","article-title":"Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene","volume":"326","year":"1992","journal-title":"N Engl J Med"},{"key":"bib5-20230925","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1016\/S1474-4422(03)00323-5","article-title":"Familial and sporadic fatal insomnia","volume":"2","year":"2003","journal-title":"Lancet Neurol"},{"key":"bib6-20230925","doi-asserted-by":"crossref","first-page":"2565","DOI":"10.1212\/WNL.43.12.2565","article-title":"[18F]FDG PET in fatal familial insomnia: the functional effects of thalamic lesions","volume":"43","year":"1993","journal-title":"Neurology"},{"key":"bib7-20230925","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1212\/WNL.49.1.126","article-title":"Cerebral metabolism in fatal familial insomnia: relation to duration, neuropathology, and distribution of protease-resistant prion protein","volume":"49","year":"1997","journal-title":"Neurology"},{"key":"bib8-20230925","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1002\/ana.21358","article-title":"Fatal familial insomnia: clinical features and early identification","volume":"63","year":"2008","journal-title":"Ann Neurol"},{"key":"bib9-20230925","doi-asserted-by":"crossref","first-page":"668","DOI":"10.1093\/brain\/awl003","article-title":"Pre-symptomatic diagnosis in fatal familial insomnia: serial neurophysiological and 18FDG-PET studies","volume":"129","year":"2006","journal-title":"Brain"}],"container-title":["Clinical Nuclear Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.lww.com\/00003072-201808000-00030","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T05:06:13Z","timestamp":1695618373000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/00003072-201808000-00030"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8]]},"references-count":9,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2018]]}},"URL":"https:\/\/doi.org\/10.1097\/rlu.0000000000002152","relation":{},"ISSN":["1536-0229","0363-9762"],"issn-type":[{"value":"1536-0229","type":"electronic"},{"value":"0363-9762","type":"print"}],"subject":[],"published":{"date-parts":[[2018,8]]}}}