{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:32:48Z","timestamp":1760146368011,"version":"build-2065373602"},"reference-count":23,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2024,10,30]],"date-time":"2024-10-30T00:00:00Z","timestamp":1730246400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009619","name":"AMED","doi-asserted-by":"publisher","award":["JP23ek0109620","23FC1007","22FC2002"],"award-info":[{"award-number":["JP23ek0109620","23FC1007","22FC2002"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Research Committee of Prion Disease and Slow Virus Infection, Ministry of Health, Labour, Welfare","award":["JP23ek0109620","23FC1007","22FC2002"],"award-info":[{"award-number":["JP23ek0109620","23FC1007","22FC2002"]}]},{"name":"Research Committee on Surveillance and Infection Control of Prion Disease, Ministry of Health, Labour, Welfare","award":["JP23ek0109620","23FC1007","22FC2002"],"award-info":[{"award-number":["JP23ek0109620","23FC1007","22FC2002"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background\/Objectives: Sporadic Creutzfeldt\u2013Jakob disease (sCJD) is a fatal neurodegenerative disorder traditionally diagnosed based on the World Health Organization (WHO) criteria in 1998. Recently, Hermann et al. proposed updated diagnostic criteria incorporating advanced biomarkers to enhance early detection of sCJD. This study aimed to evaluate the sensitivity and specificity of Hermann\u2019s criteria compared with those of the WHO criteria in a large cohort of patients suspected of prion disease in Japan. Methods: In this retrospective cohort study, we examined the new criteria using data of 2004 patients with suspected prion disease registered with the Japanese Prion Disease Surveillance (JPDS) between January 2009 and May 2023. Patients with genetic or acquired prion diseases or incomplete data necessary for the diagnostic criteria were excluded, resulting in 786 eligible cases. The sensitivity and specificity of the WHO and Hermann\u2019s criteria were calculated by comparing diagnoses with those made by the JPDS Committee. Results: Of the 786 included cases, Hermann\u2019s criteria helped identify 572 probable cases compared with 448 by the WHO criteria. The sensitivity and specificity of the WHO criteria were 96.4% and 96.6%, respectively. Hermann\u2019s criteria demonstrated a sensitivity of 99.3% and a specificity of 95.2%, indicating higher sensitivity but slightly lower specificity. Fifty-five cases were classified as \u201cdefinite\u201d by both criteria. Conclusions: The findings suggest that Hermann\u2019s criteria could offer improved sensitivity for detecting sCJD, potentially reducing diagnostic oversight. However, caution is advised in clinical practice to avoid misdiagnosis, particularly in treatable neurological diseases, by ensuring thorough exclusion of other potential conditions.<\/jats:p>","DOI":"10.3390\/diagnostics14212424","type":"journal-article","created":{"date-parts":[[2024,10,31]],"date-time":"2024-10-31T08:44:04Z","timestamp":1730364244000},"page":"2424","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["A Retrospective Cohort Study of a Newly Proposed Criteria for Sporadic Creutzfeldt\u2013Jakob Disease"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0001-9914-2599","authenticated-orcid":false,"given":"Toshiaki","family":"Nonaka","sequence":"first","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2660-530X","authenticated-orcid":false,"given":"Ryusuke","family":"Ae","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Division of Public Health, Center for Community Medicine, Jichi Medical University, Shimotsuke 329-0498, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1050-2699","authenticated-orcid":false,"given":"Koki","family":"Kosami","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Division of Public Health, Center for Community Medicine, Jichi Medical University, Shimotsuke 329-0498, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroya","family":"Tange","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miho","family":"Kaneko","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1960-8326","authenticated-orcid":false,"given":"Takehiro","family":"Nakagaki","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tsuyoshi","family":"Hamaguchi","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Kanazawa Medical University, Kahoku-gun 920-0293, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuo","family":"Sanjo","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Internal Medicine, Division of Neurology, Kudanzaka Hospital, Tokyo 102-0074, Japan"},{"name":"Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School of Medical and Dental Sciences, Tokyo 113-8519, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6654-1481","authenticated-orcid":false,"given":"Yoshikazu","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Division of Public Health, Center for Community Medicine, Jichi Medical University, Shimotsuke 329-0498, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuyuki","family":"Kitamoto","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurological Science, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiyuki","family":"Kuroiwa","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology and Stroke Center, Teikyo University School of Medicine, Mizonokuchi Hospital, Kawasaki 213-8507, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2178-0769","authenticated-orcid":false,"given":"Kensaku","family":"Kasuga","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Molecular Genetics, Brain Research Institute, Niigata University, Niigata 951-8585, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6264-9927","authenticated-orcid":false,"given":"Manabu","family":"Doyu","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Aichi Medical University, Nagakute 480-1195, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fumiaki","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology and Stroke Medicine, Yokohama City University Graduate School of Medicine, Yokohama 236-0004, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koji","family":"Abe","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"National Center of Neurology and Psychiatry (NCNP), Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Science, Okayama University, Okayama 700-8558, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeo","family":"Murayama","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Brain Bank for Neurodevelopmental, Neurological and Psychiatric Disorders, Molecular Research Center for Children\u2019s Mental Development, United Graduate School of Child Development, Osaka University, Osaka 565-0871, Japan"},{"name":"Brain Bank for Aging Research, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo 173-0015, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1734-7997","authenticated-orcid":false,"given":"Ichiro","family":"Yabe","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo 060-8638, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideki","family":"Mochizuki","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Osaka University Graduate School of Medicine, Osaka 565-0871, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4629-1312","authenticated-orcid":false,"given":"Takuya","family":"Matsushita","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Kochi Medical School, Kochi University, Nankoku 783-8505, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Murai","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, International University of Health and Welfare, Narita 286-8686, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masashi","family":"Aoki","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Tohoku University Graduate School of Medicine, Sendai 980-8574, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koji","family":"Fujita","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, Tokushima University Graduate School of Biomedical Sciences, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masafumi","family":"Harada","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Radiology, Tokushima University Graduate School, Tokushima 770-8503, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaki","family":"Takao","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Clinical Laboratory, National Centre of Neurology and Psychiatry (NCNP), Tokyo 187-8551, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadashi","family":"Tsukamoto","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Neurology, National Center of Neurology and Psychiatry (NCNP), Tokyo 187-8551, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0059-3104","authenticated-orcid":false,"given":"Yasushi","family":"Iwasaki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute 480-1195, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2416-5662","authenticated-orcid":false,"given":"Masahito","family":"Yamada","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Department of Internal Medicine, Division of Neurology, Kudanzaka Hospital, Tokyo 102-0074, Japan"},{"name":"Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School of Medical and Dental Sciences, Tokyo 113-8519, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hidehiro","family":"Mizusawa","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"National Center of Neurology and Psychiatry (NCNP), Tokyo 187-8551, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8007-8710","authenticated-orcid":false,"given":"Katsuya","family":"Satoh","sequence":"additional","affiliation":[{"name":"Japanese Prion Disease Surveillance Committee, Tokyo 187-8551, Japan"},{"name":"Unit of Medical and Dental Sciences, Department of Health Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8501, Japan"},{"name":"Department of Brain Research Unit, Leading Medical Research Core Unit, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8501, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Noriyuki","family":"Nishida","sequence":"additional","affiliation":[{"name":"Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki 852-8523, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2024,10,30]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"4540","DOI":"10.1016\/j.molcel.2021.08.011","article-title":"High-Resolution Structure and Strain Comparison of Infectious Mammalian Prions","volume":"81","author":"Kraus","year":"2021","journal-title":"Mol. 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