{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T18:53:27Z","timestamp":1759776807554,"version":"3.40.5"},"reference-count":34,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2020,11,27]],"date-time":"2020-11-27T00:00:00Z","timestamp":1606435200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>The aim of our study is to review the relationship between NCSE and sCJD. Creutzfeldt\u2013Jakob disease (CJD) is the most common form of human prion disease. Electroencephalography (EEG)\u2010detected changes such as periodic sharp wave complexes, superimposable to those seen in non\u2010convulsive epileptic status (NCSE), have only rarely been described at CJD onset, especially in sporadic CJD (sCJD) cases.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We describe clinical, EEG, cerebrospinal fluid (CSF) and neuroimaging findings of a confirmed case of sCJD with tau pathology, initially diagnosed as NCSE. We performed a literature review in PubMed of previous publications on both sCJD and NCSE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>An 82\u2010year\u2010old woman with no medical history presented with a 2\u2010week rapidly progressive neurological disorder, with motor aphasia, myoclonus, pyramidalism, and left posterior alien hand. EEG showed periodic sharp waves on right frontal regions, so anti\u2010epileptic treatment was started. CSF results were normal. Brain magnetic resonance imaging demonstrated hyperintensity of the right cerebral cortex in diffusion sequences. Due to suspected new\u2010onset refractory status epilepticus (NORSE), corticosteroid treatment was started, without clinical improvement. Necropsy results confirmed sCJD with tau pathology. The literature review identified 14 references including a total of 18 cases with NCSE as the presenting symptom of sCJD; the clinical and results in complementary tests were compiled into a table.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Sporadic CJD should be considered in the differential diagnosis of patients with rapid cognitive decline and EEG changes consistent with NCSE. The wide heterogeneity in the etiology of NCSE, including autoimmune disorders, especially NORSE, suggests immunotherapy should be initiated based on a good risk\u2013benefit balance. Some cases of sCJD, such as the present case with tau pathology, may mimic this clinico\u2010electrical course.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14624","type":"journal-article","created":{"date-parts":[[2020,11,2]],"date-time":"2020-11-02T06:39:58Z","timestamp":1604299198000},"page":"1385-1391","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Sporadic Creutzfeldt\u2013Jakob disease with tau pathology mimicking new\u2010onset refractory non\u2010convulsive status epilepticus: Case report and review of the literature"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1228-1503","authenticated-orcid":false,"given":"Alicia","family":"Gonzalez\u2010Martinez","sequence":"first","affiliation":[{"name":"Neurology Department Hospital Universitario de La Princesa Madrid 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