{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T22:37:39Z","timestamp":1776465459822,"version":"3.51.2"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,2,4]],"date-time":"2021-02-04T00:00:00Z","timestamp":1612396800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","award":["17K07097"],"award-info":[{"award-number":["17K07097"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:p>We report the general autopsy findings of abnormal prion protein (PrP) deposits with their seeding activities, as assessed by the real\u2010time quaking\u2010induced conversion (RT\u2010QuIC) method, in a 72\u2010year\u2010old female patient with sporadic Creutzfeldt\u2013Jakob disease (sCJD). At 68\u2009years of age, she presented with gait disturbance and visual disorders. Electroencephalography showed periodic synchronous discharge. Myoclonus was also observed. A genetic test revealed that <jats:italic>PRNP<\/jats:italic> codon 129 was methionine\/methionine (MM). She died of pneumonia three\u2009years and four\u2009months after disease onset, and a general autopsy was performed. The brain weighed 650 g and appeared markedly atrophic. Immunohistochemistry for PrP revealed synaptic PrP deposits and coarse PrP deposits in the cerebral cortices, basal ganglia, cerebellum, and brainstem. Western blot analysis identified type 1 proteinase\u2010K\u2010resistant PrP in frontal cortex samples. PrP deposits were also observed in systemic organs, including the femoral nerve, psoas major muscle, abdominal skin, adrenal medulla, zona reticularis of the adrenal gland, islet cells of the pancreas, and thyroid gland. The RT\u2010QuIC method revealed positive seeding activities in all examined organs, including the frontal cortex, femoral nerve, psoas major muscle, scalp, abdominal skin, adrenal gland, pancreas, and thyroid gland. The following 50% seeding dose (SD<jats:sub>50<\/jats:sub>) values were 9.5 (frontal cortex); 8 \u00b1 0.53 (femoral nerve); 7 \u00b1 0.53 (psoas major muscle); and 7.88 \u00b1 0.17 (scalp). The SD<jats:sub>50<\/jats:sub> values for the adrenal gland, dermis, pancreas, and thyroid gland were 6.12\u2009\u00b1\u20090.53, 5.25, 4.75, and 4.5, respectively. PrP deposits in general organs may be associated with long\u2010term disease duration. This case indicated the necessity for general autopsies in sCJD cases to establish strict infection control procedures for surgical treatment and to examine certain organs.<\/jats:p>","DOI":"10.1111\/neup.12717","type":"journal-article","created":{"date-parts":[[2021,2,8]],"date-time":"2021-02-08T02:16:50Z","timestamp":1612750610000},"page":"152-158","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Abnormal prion protein deposits with high seeding activities in the skeletal muscle, femoral nerve, and scalp of an autopsied case of sporadic Creutzfeldt\u2013Jakob disease"],"prefix":"10.1111","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4225-7248","authenticated-orcid":false,"given":"Hiroyuki","family":"Honda","sequence":"first","affiliation":[{"name":"Department of Neuropathology Kyushu University  Fukuoka 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and Immunology Nagasaki University Graduate School of Biomedical Sciences  Nagasaki Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7826-870X","authenticated-orcid":false,"given":"Toru","family":"Iwaki","sequence":"additional","affiliation":[{"name":"Department of Neuropathology Kyushu University  Fukuoka 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