{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T13:17:57Z","timestamp":1786627077707,"version":"build-2736575974"},"reference-count":43,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,1,4]],"date-time":"2025-01-04T00:00:00Z","timestamp":1735948800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Contemporary studies report nonconvulsive status epilepticus (NCSE) in Creutzfeldt\u2013Jakob disease (CJD), based on benzodiazepine (BZP)\u2010responsive epileptiform discharges on the electroencephalogram (EEG), with the following false syllogism: (1) intravenous (IV) administration of BZPs usually suppress ictal activity in NCSE; (2) in CJD, periodic sharp wave complexes (PSWCs) are suppressed by IV BZPs; (3) therefore, these patients have NCSE. This is a simplistic and invalid conclusion, because authors of 20th\u2010century science reports have clearly shown that IV BZPs, short\u2010acting barbiturates, and drugs with no antiseizure effects, such as chloral hydrate and IV naloxone, suppress PSWCs, but patients fall asleep with no clinical improvement. In contrast, IV methylphenidate transiently improves both the EEG and clinical states. Unlike NCSE, which is unlikely to be stopped by external stimuli, PSWCs can be transiently stopped by sensory or painful stimulation. Since the end of the 1970s, the effect of spontaneous sleep on the disappearance of PSWCs has been well documented, with a description of a cycling alternating pattern. Phase A features periodic discharges and is associated with increased arousal, whereas phase B exhibits a reduction or suppression of the PSWCs and is associated with a reduction in the arousal level and hypotonia (non\u2010rapid eye movement sleep). When considering the use of IV BZP administration during EEG as a diagnostic test, the sequence of disappearance of PSWCs at sleep onset and reappearance after each stimulation or sleep apnea episode is compelling evidence against NCSE, as is the observation of a pattern of stimulus\u2010induced wakefulness with transient improvement of the EEG. The cycling alternating pattern during sleep and reactivity to sensory or painful stimulation disappear with increasing disease severity; however, this occurs in the later stages of the disease, where there is no diagnostic doubt.<\/jats:p>","DOI":"10.1111\/epi.18259","type":"journal-article","created":{"date-parts":[[2025,1,4]],"date-time":"2025-01-04T09:24:48Z","timestamp":1735982688000},"page":"1006-1013","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["When patients with Creutzfeldt\u2013Jakob disease are misdiagnosed as having nonconvulsive status epilepticus"],"prefix":"10.1111","volume":"66","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9296-1957","authenticated-orcid":false,"given":"Philippe","family":"G\u00e9lisse","sequence":"first","affiliation":[{"name":"Epilepsy Unit H\u00f4pital Gui de Chauliac  Montpellier France"},{"name":"Research Unit, Unit\u00e9 de Recherche sur les Comportements et Mouvements Anormaux Institut national de la sant\u00e9 et de la recherche m\u00e9dicale (INSERM)  Montpellier France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2203-1938","authenticated-orcid":false,"given":"Arielle","family":"Crespel","sequence":"additional","affiliation":[{"name":"Epilepsy Unit H\u00f4pital Gui de Chauliac  Montpellier France"},{"name":"Research Unit, Unit\u00e9 de Recherche sur les Comportements et Mouvements Anormaux Institut national de la sant\u00e9 et de la recherche m\u00e9dicale (INSERM)  Montpellier 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