{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T07:33:54Z","timestamp":1761896034322},"reference-count":24,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,3,18]],"date-time":"2015-03-18T00:00:00Z","timestamp":1426636800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Austrian Federal Ministry of Health (BMG)"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2015,8]]},"abstract":"<jats:p>We describe an atypical neuropathological phenotype of sporadic <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>reutzfeldt\u2010<jats:styled-content style=\"fixed-case\">J<\/jats:styled-content>akob disease in a 76\u2010year\u2010old man. The clinical symptoms were characterized by progressive dementia, gait ataxia, rigidity and urinary incontinence. The disease duration was 6 weeks. <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> did not show prominent atrophy or hyperintensities in cortical areas, striatum or thalamus. Biomarker examination of the cerebrospinal fluid deviated from that seen in pure <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>lzheimer's disease. Triphasic waves in the <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content> were detected only later in the disease course, while 14\u20103\u20103 assay was positive. <jats:italic><jats:styled-content style=\"fixed-case\">PRNP<\/jats:styled-content><\/jats:italic> genotyping revealed methionine homozygosity (<jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content>) at codon 129. Neuropathology showed classical <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> changes corresponding to the <jats:styled-content style=\"fixed-case\">MM<\/jats:styled-content> type 1 cases. However, a striking feature was the presence of abundant kuru\u2010type plaques in the white matter. This rare morphology was associated with neuropathological signs of intranuclear inclusion body disease and advanced stage of argyrophilic grain disease. These alterations did not show correlation with each other, thus seemed to develop independently. This case further highlights the complexity of neuropathological alterations in the ageing brain.<\/jats:p>","DOI":"10.1111\/neup.12192","type":"journal-article","created":{"date-parts":[[2015,3,18]],"date-time":"2015-03-18T07:51:47Z","timestamp":1426665107000},"page":"336-342","source":"Crossref","is-referenced-by-count":12,"title":["Atypical sporadic <scp>CJD\u2010MM<\/scp> phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease"],"prefix":"10.1111","volume":"35","author":[{"given":"Anna S.","family":"Berghoff","sequence":"first","affiliation":[{"name":"Institute of Neurology and Austrian Reference Center for Human Prion Diseases Medical University of Vienna  Vienna Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anita","family":"Trummert","sequence":"additional","affiliation":[{"name":"Landesklinikum Weinviertel  Mistelbach\u2010G\u00e4nserndorf 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