{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T08:58:14Z","timestamp":1762160294849},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2004,10,8]],"date-time":"2004-10-08T00:00:00Z","timestamp":1097193600000},"content-version":"vor","delay-in-days":5943,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[1988,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The older brother of the patient from whom the Fukuoka\u2010l strain was isolated was found to have numerous kuru plaques, the main finding common to both siblings. Other clinicopathological features including spongiform change were absent in the older brother. Immunostaining using anti\u2010kuru plaque core protein and anti\u2010<jats:sup>\u03b2<\/jats:sup>\u2010protein peptide revealed many kuru plaques and a few senile plaques in the older brother. Experimental transmission of the disease to laboratory animals was successful, using tissues from both siblings, through inoculation of fresh brain homogenates, purified prion protein, and formalin\u2010fixed brain homogenates. Prion protein fractions from the patient's brain shortened the incubation periods and formalin\u2010fixed mouse brains did not lengthen the periods. The disease in the two brothers can be classified as Gerstmann\u2010Str\u00e4ussler\u2010Scheinker disease, a familial variant of Creutzfeldt\u2010Jakob disease. Gerstmann\u2010Str\u00e4ussler\u2010Scheinker disease manifests a variety of clinicopathological features. Immunohistological verification of kuru plaques has major diagnostic value in assessing dementia.<\/jats:p>","DOI":"10.1002\/ana.410240108","type":"journal-article","created":{"date-parts":[[2005,1,1]],"date-time":"2005-01-01T14:01:38Z","timestamp":1104588098000},"page":"35-40","source":"Crossref","is-referenced-by-count":39,"title":["Gerstmann\u2010Str\u00e4ussler\u2010Scheinker disease: Immunohistological and experimental studies"],"prefix":"10.1002","volume":"24","author":[{"given":"June","family":"Tateishi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuyuki","family":"Kitamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideyuki","family":"Hashiguchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirofumi","family":"Shii","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,10,8]]},"reference":[{"key":"e_1_2_1_2_2","first-page":"182","volume-title":"Handbook of clinical neurology","author":"Seitelberger F","year":"1981"},{"key":"e_1_2_1_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00707108"},{"key":"e_1_2_1_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/104.3.559"},{"key":"e_1_2_1_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410050616"},{"key":"e_1_2_1_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410070424"},{"key":"e_1_2_1_7_2","doi-asserted-by":"crossref","first-page":"1050","DOI":"10.1128\/iai.37.3.1050-1053.1982","article-title":"Evidence for an unconventional virus in mouse adapted Creutzfeldt\u2010Jakob disease","volume":"37","author":"Kingsbury DT","year":"1982","journal-title":"Infect Immun"},{"key":"e_1_2_1_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(84)80190-4"},{"key":"e_1_2_1_9_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.82.12.4245"},{"key":"e_1_2_1_10_2","doi-asserted-by":"crossref","unstructured":"BoltonDC McKinleyMP PrusinerSB. 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