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000139709 0247_ $$2doi$$a10.1016/j.seizure.2017.10.023
000139709 0247_ $$2pmid$$apmid:29125945
000139709 0247_ $$2ISSN$$a1059-1311
000139709 0247_ $$2ISSN$$a1532-2688
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000139709 037__ $$aDZNE-2020-06031
000139709 041__ $$aEnglish
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000139709 1001_ $$0P:(DE-HGF)0$$aMarquetand, Justus$$b0$$eCorresponding author
000139709 245__ $$aPeriodic EEG patterns in sporadic Creutzfeld-Jakob-Disease can be benzodiazepine-responsive and be difficult to distinguish from non-convulsive status epilepticus.
000139709 260__ $$aOxford [u.a.]$$bElsevier$$c2017
000139709 264_1 $$2Crossref$$3print$$bElsevier BV$$c2017-12-01
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000139709 520__ $$aPeriodic discharges in EEG and a history of rapidly progressive dementia are known to be associated with sporadic Creutzfeld-Jakob Disease (sCJD). Doubts regarding this rare but fatal diagnosis can arise, when episodic symptoms (seizures, psychiatric features, speech disturbances) are present and the EEG shows epileptiform discharges within this periodicity. This scenario may indicate non-convulsive status epilepticus (NCSE), which is - in contrast to sCJD - a treatable and frequent condition.Herein we report a small retrospective study of 4 elderly patients, who suffered from sCJD, but due to episodic symptoms and benzodiazepine-responsive epileptiform discharges in combination to a subacute cognitive decline, NCSE was considered as a potential differential diagnosis and therefore treated aggressively.Ultimately, this treatment was not successful and sCJD was diagnosed in all cases. Hence, there was no diagnostic and prognostic value of abolishing periodic discharges in EEG via benzodiazepines in differentiating sCJD from NCSE in our series.These findings indicate that periodic discharges in sCJD can be responsive to benzodiazepines andnot necessarily help to differentiate differentiation between sCJD and NCSE of other causes. We argue that an aggressive anticonvulsive treatment in this scenario should be considered cautiously, especially for invasive options like general anesthesia.
000139709 536__ $$0G:(DE-HGF)POF3-345$$a345 - Population Studies and Genetics (POF3-345)$$cPOF3-345$$fPOF III$$x0
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000139709 542__ $$2Crossref$$i2018-12-01$$uhttp://www.elsevier.com/open-access/userlicense/1.0/
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000139709 650_7 $$012794-10-4$$2NLM Chemicals$$aBenzodiazepines
000139709 650_2 $$2MeSH$$aAged
000139709 650_2 $$2MeSH$$aBenzodiazepines: administration & dosage
000139709 650_2 $$2MeSH$$aBenzodiazepines: pharmacology
000139709 650_2 $$2MeSH$$aCognitive Dysfunction: diagnosis
000139709 650_2 $$2MeSH$$aCreutzfeldt-Jakob Syndrome: diagnosis
000139709 650_2 $$2MeSH$$aDiagnosis, Differential
000139709 650_2 $$2MeSH$$aElectroencephalography: methods
000139709 650_2 $$2MeSH$$aFemale
000139709 650_2 $$2MeSH$$aHumans
000139709 650_2 $$2MeSH$$aMale
000139709 650_2 $$2MeSH$$aRetrospective Studies
000139709 650_2 $$2MeSH$$aStatus Epilepticus: diagnosis
000139709 650_2 $$2MeSH$$aStatus Epilepticus: drug therapy
000139709 7001_ $$0P:(DE-HGF)0$$aKnake, Susanne$$b1
000139709 7001_ $$0P:(DE-HGF)0$$aStrzelczyk, Adam$$b2
000139709 7001_ $$0P:(DE-HGF)0$$aSteinhoff, Bernhard J$$b3
000139709 7001_ $$0P:(DE-HGF)0$$aLerche, Holger$$b4
000139709 7001_ $$0P:(DE-2719)2811275$$aSynofzik, Matthis$$b5$$udzne
000139709 7001_ $$0P:(DE-HGF)0$$aFocke, Niels K$$b6
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