{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,11]],"date-time":"2026-06-11T14:29:48Z","timestamp":1781188188309,"version":"3.54.1"},"reference-count":21,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,9,3]],"date-time":"2014-09-03T00:00:00Z","timestamp":1409702400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropathology"],"published-print":{"date-parts":[[2015,2]]},"abstract":"<jats:p>We report hereby an autopsy case of sporadic mixed phenotype <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> without hereditary burden and a long\u2010term clinical course. An 80\u2010year old man was diagnosed with mild cognitive impairment 27 months before death, caused by bronchopneumonia and severe respiratory impairment. During this time, the patient developed gradual mental deterioration, some sleeping problems and myoclonus. Other clinical manifestations were progressive gait problems, language deterioration, presence of primitive reflexes and irritability. In keeping with those symptoms, a rapidly evolving dementia was clinically suspected. Cerebrospinal fluid test for 14\u20103\u20103 protein was negative. However, an abnormal <jats:styled-content style=\"fixed-case\">EEG<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> at end\u2010stage of disease were finally consistent with <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content>. Post\u2010mortem examination revealed a massive cortical neuronal loss with associated reactive astrocytosis, also evident in the white matter. Diffuse spongiform changes involving some basal ganglia, especially medial thalamus, some troncoencephalic nuclei, mainly inferior olivary nucleus and the molecular layer of the cerebellum were seen. Immunorreactive deposits for anti\u2010prion protein antibody were present at different areas of the <jats:styled-content style=\"fixed-case\">CNS<\/jats:styled-content>. Additionally, <jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>ewy bodies were observed at the brainstem and amygdala. Furthermore, argirophilic grains together with oligodendroglial coiled bodies and pre\u2010tangle inclusions in the neurons from the limbic system containing hyperphosphorylated <jats:styled-content style=\"fixed-case\">4R<\/jats:styled-content> tau were noted. To the best of our knowledge, this is the first case of <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> combined with <jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>ewy body disease and argirophilic grain disease. Furthermore, we believe this case is an extremely rare combination of <jats:styled-content style=\"fixed-case\">MM2<\/jats:styled-content>\u2010cortical\u2010type and <jats:styled-content style=\"fixed-case\">MM2<\/jats:styled-content>\u2010thalamic\u2010type sporadic <jats:styled-content style=\"fixed-case\">CJD<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">sCJD<\/jats:styled-content>), which explains the broad spectrum of <jats:styled-content style=\"fixed-case\">MM2<\/jats:styled-content>\u2010type <jats:styled-content style=\"fixed-case\">sCJD<\/jats:styled-content> findings and symptoms. Moreover, histological features of possible <jats:styled-content style=\"fixed-case\">A<\/jats:styled-content>lzheimer's disease were also reported.<\/jats:p>","DOI":"10.1111\/neup.12150","type":"journal-article","created":{"date-parts":[[2014,9,3]],"date-time":"2014-09-03T08:27:16Z","timestamp":1409732836000},"page":"56-63","source":"Crossref","is-referenced-by-count":16,"title":["Coexistence of mixed phenotype <scp>C<\/scp>reutzfeldt\u2010<scp>J<\/scp>akob disease, <scp>L<\/scp>ewy body disease and argyrophilic grain disease plus histological features of possible <scp>A<\/scp>lzheimer's disease: A multi\u2010protein disorder in an autopsy case"],"prefix":"10.1111","volume":"35","author":[{"given":"Iv\u00e1n","family":"Fern\u00e1ndez\u2010Vega","sequence":"first","affiliation":[{"name":"Pathology Department Hospital Universitario Araba  \u00c1lava Spain"},{"name":"Biobanco Vasco para la Investigaci\u00f3n (O+eHun) Brain Bank Hospital Universitario Araba  \u00c1lava Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Javier","family":"Ruiz\u2010Ojeda","sequence":"additional","affiliation":[{"name":"Neurology Department Hospital de Galdakao\u2010Usansolo  Bizkaia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramon A.","family":"Juste","sequence":"additional","affiliation":[{"name":"Department of Animal Health NEIKER\u2010Tecnalia  Bizkaia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Geijo","sequence":"additional","affiliation":[{"name":"Department of Animal Health NEIKER\u2010Tecnalia  Bizkaia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan Jose","family":"Zarranz","sequence":"additional","affiliation":[{"name":"Neurology Department Hospital Universitario de Cruces  Bizkaia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose Luis","family":"S\u00e1nchez Menoyo","sequence":"additional","affiliation":[{"name":"Neurology Department Hospital de Galdakao\u2010Usansolo  Bizkaia Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ikerne","family":"Vicente\u2010Etxenausia","sequence":"additional","affiliation":[{"name":"Biobanco Vasco para la Investigaci\u00f3n (O+eHun) Brain Bank Hospital Universitario Araba  \u00c1lava Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Mediavilla\u2010Garc\u00eda","sequence":"additional","affiliation":[{"name":"Biobanco Vasco para la Investigaci\u00f3n (O+eHun) Brain Bank Hospital Universitario Araba  \u00c1lava Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabel","family":"Guerra\u2010Merino","sequence":"additional","affiliation":[{"name":"Pathology Department Hospital Universitario Araba  \u00c1lava Spain"},{"name":"Biobanco Vasco para la Investigaci\u00f3n (O+eHun) Brain Bank Hospital Universitario Araba  \u00c1lava 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