TY  - JOUR
AU  - Marquetand, Justus
AU  - Knake, Susanne
AU  - Strzelczyk, Adam
AU  - Steinhoff, Bernhard J
AU  - Lerche, Holger
AU  - Synofzik, Matthis
AU  - Focke, Niels K
TI  - Periodic EEG patterns in sporadic Creutzfeld-Jakob-Disease can be benzodiazepine-responsive and be difficult to distinguish from non-convulsive status epilepticus.
JO  - Seizure
VL  - 53
SN  - 1059-1311
CY  - Oxford [u.a.]
PB  - Elsevier
M1  - DZNE-2020-06031
SP  - 47-50
PY  - 2017
AB  - Periodic 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.
KW  - Aged
KW  - Benzodiazepines: administration & dosage
KW  - Benzodiazepines: pharmacology
KW  - Cognitive Dysfunction: diagnosis
KW  - Creutzfeldt-Jakob Syndrome: diagnosis
KW  - Diagnosis, Differential
KW  - Electroencephalography: methods
KW  - Female
KW  - Humans
KW  - Male
KW  - Retrospective Studies
KW  - Status Epilepticus: diagnosis
KW  - Status Epilepticus: drug therapy
KW  - Benzodiazepines (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:29125945
DO  - DOI:10.1016/j.seizure.2017.10.023
UR  - https://pub.dzne.de/record/139709
ER  -