{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T18:26:21Z","timestamp":1792002381437,"version":"4.4.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T00:00:00Z","timestamp":1714953600000},"content-version":"vor","delay-in-days":5,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Department of Health and Social Care Policy Research Programme"},{"DOI":"10.13039\/100012095","name":"Scottish Government","doi-asserted-by":"publisher","award":["PR-ST-0614-00008_18"],"award-info":[{"award-number":["PR-ST-0614-00008_18"]}],"id":[{"id":"10.13039\/100012095","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,5,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Introduction<\/jats:title>\n                  <jats:p>Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease with public health implications. Mean age of onset is 68\u00a0years. Age-specific incidence declines after 80\u00a0years. This may arise from under-ascertainment or other biological features of the disease. Accurate characterisation of late-onset sCJD is important for early diagnosis, avoiding unnecessary investigations and improving ascertainment for public health purposes.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To phenotype the clinical features and investigation profile of sCJD in adults &amp;gt;80\u00a0years.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We analysed all probable and definite sCJD cases identified by the UK National CJD Research &amp; Surveillance Unit over a 10-year period (2011\u20132021). Individuals were grouped by age of onset. Clinical features and investigation profiles were compared.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>10.3% (123\/1196) had an age of onset over 80. Median survival was shorter (3.2 vs 4.3\u00a0months; P\u2009&amp;lt;\u20090.001). Pyramidal signs (48.3% vs 34.2%; P\u2009=\u20090.008) and akinetic mutism (55.1% vs 33.2%; P\u2009&amp;lt;\u20090.001) were more frequent. Psychiatric symptoms (26.3% vs 39.6%; P\u2009=\u20090.01) and cerebellar signs (65.4% vs 78.6%, P\u2009=\u20090.007) were less frequent. Cognitive impairment and myoclonus were highly prevalent regardless of age. Between age groups, the diagnostic sensitivity of cerebrospinal fluid real-time quaking-induced conversion (CSF RT-QuIC) (92.9% vs 91.9%, P\u2009=\u20090.74) was comparable, electroencephalography was superior (41.5% vs 25.4%; P\u2009=\u20090.006) and MRI was inferior (67.8% vs 91.4%; P\u2009&amp;lt;\u20090.001).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>Late-onset sCJD has distinct clinical features, shorter survival and a different profile of investigation sensitivity. CSF RT-QuIC, MRI brain and specialist CJD review is recommended in older adults with a rapidly progressive neurological disorder. Autopsy is valuable when the cause remains elusive.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ageing\/afae086","type":"journal-article","created":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T06:57:11Z","timestamp":1714978631000},"source":"Crossref","is-referenced-by-count":10,"title":["Sporadic Creutzfeldt-Jakob disease in adults over 80\u00a0years: a 10-year review of United Kingdom surveillance"],"prefix":"10.1093","volume":"53","author":[{"given":"Eugene Ace","family":"McDermott","sequence":"first","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil","family":"Watson","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johnny","family":"Tam","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Centola","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hatice","family":"Kurucu King","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Mackenzie","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Summers","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Green","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcelo A","family":"Barria","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colin","family":"Smith","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suvankar","family":"Pal","sequence":"additional","affiliation":[{"name":"The National CJD Research & Surveillance Unit , Centre for Clinical Brain Sciences, , Edinburgh , UK"},{"name":"University of Edinburgh , Centre for Clinical Brain Sciences, , Edinburgh , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2024,5,5]]},"reference":[{"key":"2024050606570604600_ref1","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1038\/s41582-021-00488-7","article-title":"The importance of ongoing international surveillance for Creutzfeldt\u2013Jakob disease","volume":"17","author":"Watson","year":"2021","journal-title":"Nat Rev Neurol"},{"key":"2024050606570604600_ref2","doi-asserted-by":"crossref","first-page":"e2","DOI":"10.1016\/S1473-3099(19)30615-2","article-title":"Review Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation","volume":"20","author":"Uttley","year":"2020","journal-title":"Lancet Infect Dis"},{"key":"2024050606570604600_ref3","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W","article-title":"Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects","volume":"46","author":"Parchi","year":"1999","journal-title":"Ann Neurol"},{"key":"2024050606570604600_ref4","volume-title":"Sporadic CJD: Annual Mortality Rates per Million","author":"Creutzfeldt-Jakob Disease International Surveillance Network","year":"2021"},{"key":"2024050606570604600_ref5","volume-title":"Creutzfeldt-Jakob Disease Surveillance in the UK.","author":"The National CJD Research & Surveillance Unit","year":"2022"},{"key":"2024050606570604600_ref6","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1136\/jnnp-2022-330153","article-title":"Evaluation of the impact of CSF prion RT-QuIC and amended criteria on the clinical diagnosis of Creutzfeldt-Jakob disease: a 10-year study in Italy","volume":"94","author":"Mastrangelo","year":"2023","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2024050606570604600_ref7","doi-asserted-by":"crossref","first-page":"e331","DOI":"10.1212\/WNL.0000000000005860","article-title":"Validation and utilization of amended diagnostic criteria in Creutzfeldt-Jakob disease surveillance","volume":"91","author":"Hermann","year":"2018","journal-title":"Neurology"},{"key":"2024050606570604600_ref8","doi-asserted-by":"crossref","first-page":"e2146319","DOI":"10.1001\/jamanetworkopen.2021.46319","article-title":"Validation of revised international Creutzfeldt-Jakob disease surveillance network diagnostic criteria for sporadic Creutzfeldt-Jakob disease","volume":"5","author":"Watson","year":"2022","journal-title":"JAMA Netw Open"},{"key":"2024050606570604600_ref9","doi-asserted-by":"crossref","first-page":"111","DOI":"10.31887\/DCNS.2009.11.2\/cqiu","article-title":"Epidemiology of Alzheimer\u2019s disease: occurrence, determinants, and strategies toward intervention","volume":"11","author":"Qiu","year":"2009","journal-title":"Dialogues Clin Neurosci"},{"key":"2024050606570604600_ref10","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1038\/s41531-022-00280-4","article-title":"A nationwide study of the incidence, prevalence and mortality of Parkinson\u2019s disease in the Norwegian population","volume":"8","author":"Brakedal","year":"2022","journal-title":"NPJ Parkinsons Dis"},{"key":"2024050606570604600_ref11","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-020-72519-0","article-title":"A nationwide trend analysis in the incidence and mortality of Creutzfeldt\u2013Jakob disease in Japan between 2005 and 2014","volume":"10","author":"Nishimura","year":"2020","journal-title":"Sci Rep"},{"key":"2024050606570604600_ref12","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1093\/brain\/awm063","article-title":"Creutzfeldt-Jakob disease in Germany: a prospective 12-year surveillance","volume":"130","author":"Heinemann","year":"2007","journal-title":"Brain"},{"key":"2024050606570604600_ref13","doi-asserted-by":"crossref","first-page":"1736","DOI":"10.1212\/WNL.0000000000002638","article-title":"Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes","volume":"86","author":"Coyle-Gilchrist","year":"2016","journal-title":"Neurology"},{"key":"2024050606570604600_ref14","doi-asserted-by":"crossref","first-page":"363","DOI":"10.1080\/21678421.2021.2016837","article-title":"Analysis of incidence of motor neuron disease in England 1998\u20132019: use of three linked datasets","volume":"23","author":"Burchardt","year":"2022","journal-title":"Amyotroph Lateral Scler Frontotemporal Degener"},{"key":"2024050606570604600_ref15","volume-title":"Protocol","author":"The National CJD Research & Surveillance 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Center","volume":"261","author":"Karch","year":"2014","journal-title":"J Neurol"},{"key":"2024050606570604600_ref27","doi-asserted-by":"crossref","first-page":"4275","DOI":"10.1007\/s10072-022-05929-9","article-title":"Epidemiological and clinical characteristics of patients with late-onset Creutzfeldt-Jakob disease","volume":"43","author":"Trachtenbroit","year":"2022","journal-title":"Neurol Sci"},{"key":"2024050606570604600_ref28","doi-asserted-by":"crossref","first-page":"428","DOI":"10.1176\/jnp.2007.19.4.428","article-title":"Does the presentation of Creutzfeldt-Jakob disease vary by age or presumed etiology? A meta-analysis of the past 10 years","volume":"19","author":"Appleby","year":"2007","journal-title":"J Neuropsychiatry Clin Neurosci"},{"key":"2024050606570604600_ref29","doi-asserted-by":"crossref","first-page":"2278","DOI":"10.1093\/brain\/awl159","article-title":"Determinants of diagnostic investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease","volume":"129","author":"Collins","year":"2006","journal-title":"Brain"},{"key":"2024050606570604600_ref30","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1093\/bmb\/66.1.213","article-title":"Sporadic and familial CJD: classification and characterisation","volume":"66","author":"Gambetti","year":"2003","journal-title":"Br Med Bull"},{"key":"2024050606570604600_ref31","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1007\/s004150050204","article-title":"The Medical Research Council prion disease rating scale: a new outcome measure for prion disease therapeutic trials developed and validated using systematic observational studies","volume":"245","author":"Thompson","journal-title":"J Neurol"},{"key":"2024050606570604600_ref32","doi-asserted-by":"crossref","first-page":"461","DOI":"10.1136\/jnnp-2017-316853","article-title":"Imaging and CSF analyses effectively distinguish CJD from its mimics","volume":"89","author":"Rudge","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry"}],"container-title":["Age and Ageing"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ageing\/article-pdf\/53\/5\/afae086\/57405237\/afae086.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ageing\/article-pdf\/53\/5\/afae086\/57405237\/afae086.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,6]],"date-time":"2024-05-06T06:57:35Z","timestamp":1714978655000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ageing\/article\/doi\/10.1093\/ageing\/afae086\/7665131"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,5,1]]},"references-count":32,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2024,5,1]]}},"URL":"https:\/\/doi.org\/10.1093\/ageing\/afae086","relation":{},"ISSN":["0002-0729","1468-2834"],"issn-type":[{"value":"0002-0729","type":"print"},{"value":"1468-2834","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,5]]},"published":{"date-parts":[[2024,5,1]]},"article-number":"afae086"}}