{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T15:53:01Z","timestamp":1775836381434,"version":"3.50.1"},"reference-count":13,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2011,6,21]],"date-time":"2011-06-21T00:00:00Z","timestamp":1308614400000},"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":[[2012,4]]},"abstract":"<jats:p>Multiple system atrophy (MSA) is a sporadic alpha\u2010synucleinopathy clinically characterized by variable degrees of parkinsonism, cerebellar ataxia and autonomic dysfunction. The histopathological hallmark of MSA is glial cytoplasmic inclusion (GCI). It is considered to represent the earliest stage of the degenerative process in MSA and to precede neuronal degeneration. Sporadic Creutzfeldt\u2010Jakob disease (sCJD) is a fatal, rapidly progressive dementia generally associated with ataxia, pyramidal and extrapyramidal symptoms and myoclonus. Definite diagnosis needs neuropathological demonstration of variable degrees of spongiform degeneration of neuropil, neuronal loss, astro\u2010 and microgliosis, and the presence of abnormal deposits of the misfolded prion protein PrP<jats:sup>res<\/jats:sup>. Both diseases, CJD and MSA are infrequent among neurodegenerative diseases. In the present report we describe clinical and neuropathological findings of a previously healthy 64\u2010year\u2010old woman who developed symptoms of classical CJD. At post mortem examination, the brain showed in addition to classical methionine\/methionine PrP<jats:sup>res<\/jats:sup> type 1 (MM1) sCJD changes and moderate Alzheimer\u2010type pathology, features of \u201cpreclinical\u201d MSA with minimal histopathological changes. These were characterized by discrete amounts of alpha\u2010synuclein immunoreacive glial cytoplasmic inclusions in the striato\u2010nigral system, isolated intraneuronal inclusions in pigmented neurons of the substantia nigra, as well as some vermiform intranuclear inclusions. To our knowledge, this is the first report on the coexistence of definite sCJD and \u201cminimal changes\u201d MSA in the same patient.<\/jats:p>","DOI":"10.1111\/j.1440-1789.2011.01232.x","type":"journal-article","created":{"date-parts":[[2011,6,22]],"date-time":"2011-06-22T02:28:17Z","timestamp":1308709697000},"page":"158-163","source":"Crossref","is-referenced-by-count":19,"title":["\u201cPreclinical\u201d MSA in definite Creutzfeldt\u2010Jakob disease"],"prefix":"10.1111","volume":"32","author":[{"given":"Roberta","family":"Rodriguez\u2010Diehl","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Jesus","family":"Rey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexandre","family":"Gironell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Martinez\u2010Saez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isidre","family":"Ferrer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raquel","family":"S\u00e1nchez\u2010Valle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jordi","family":"Jag\u00fce","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos","family":"Nos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ellen","family":"Gelpi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2011,6,21]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.58.2.160"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1440-1789.2006.00713.x"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.5414\/NPP26276"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-004-0935-y"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-008-0398-7"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awp191"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.2007.00907.x"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.20537"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-1029-1"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-0022-z"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0490-10.2010"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0713-y"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10286-007-0447-7"}],"container-title":["Neuropathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1440-1789.2011.01232.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1440-1789.2011.01232.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,14]],"date-time":"2023-10-14T02:35:14Z","timestamp":1697250914000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1440-1789.2011.01232.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,6,21]]},"references-count":13,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2012,4]]}},"alternative-id":["10.1111\/j.1440-1789.2011.01232.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1440-1789.2011.01232.x","archive":["Portico"],"relation":{},"ISSN":["0919-6544","1440-1789"],"issn-type":[{"value":"0919-6544","type":"print"},{"value":"1440-1789","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,6,21]]}}}