{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T18:14:36Z","timestamp":1791137676800,"version":"4.1.0"},"reference-count":45,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2004,10,7]],"date-time":"2004-10-07T00:00:00Z","timestamp":1097107200000},"content-version":"vor","delay-in-days":9380,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1979,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The worldwide epidemiology of Creutzfeldt\u2010Jakob disease (CJD) is presented from an analysis of 1,435 patients. In the United States, the average annual mortality rate is at least 0.26 deaths per million. Temporal\u2010spatial clustering of cases was not found in the United States, but reports from other countries indicate that such clustering does occur. Fifteen percent of the cases were of the familial type, suggesting a genetic susceptibility to infection.<\/jats:p><jats:p>Iatrogenic transmission by corneal transplantation and neurosurgical operations has occurred, and the possibility is raised that previous surgery or preexisting neurological disease may be associated with an increased risk of developing CJD. It remains to be determined whether the virus of CJD is maintained only by patient\u2010to\u2010patient transmission, has a zoonotic reservoir such as scrapie, or causes widespread latent infection of man that is occasionally activated.<\/jats:p>","DOI":"10.1002\/ana.410050212","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T16:50:46Z","timestamp":1104598246000},"page":"177-188","source":"Crossref","is-referenced-by-count":460,"title":["Creutzfeldt\u2010Jakob disease: Patterns of worldwide occurrence and the significance of familial and sporadic clustering"],"prefix":"10.1002","volume":"5","author":[{"given":"Colin L.","family":"Masters","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan O.","family":"Harris","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. Carleton","family":"Gajdusek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr","given":"Clarence J.","family":"Gibbs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Bernoulli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David M.","family":"Asher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2004,10,7]]},"reference":[{"key":"e_1_2_1_2_2","series-title":"International Congress Series No 274","first-page":"1221","volume-title":"Psychiatry. 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Retrospective study of the Paris area during the ten\u2010year period 1968\u20131977.Ann Neurol pp189\u2013192this issue","DOI":"10.1002\/ana.410050213"},{"key":"e_1_2_1_9_2","first-page":"5","article-title":"Intrahuclear corpuscles in the neurons of the substantia nigra. Anatomoclinical report of 11 cases of Jakob\u2010Creutzfeldt disease","volume":"35","author":"Cartier L","year":"1977","journal-title":"Neurocirugia (Chile)"},{"key":"e_1_2_1_10_2","unstructured":"CathalaF BrownP LhermitteF et al:Epid\u00e9miologie de la maladie de Creutzfeldt\u2010Jakob dans la r\u00e9gion Parisienne(1). 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