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@ARTICLE{Marquetand:139709,
      author       = {Marquetand, Justus and Knake, Susanne and Strzelczyk, Adam
                      and Steinhoff, Bernhard J and Lerche, Holger and Synofzik,
                      Matthis and Focke, Niels K},
      title        = {{P}eriodic {EEG} patterns in sporadic
                      {C}reutzfeld-{J}akob-{D}isease can be
                      benzodiazepine-responsive and be difficult to distinguish
                      from non-convulsive status epilepticus.},
      journal      = {Seizure},
      volume       = {53},
      issn         = {1059-1311},
      address      = {Oxford [u.a.]},
      publisher    = {Elsevier},
      reportid     = {DZNE-2020-06031},
      pages        = {47-50},
      year         = {2017},
      abstract     = {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.},
      keywords     = {Aged / Benzodiazepines: administration $\&$ dosage /
                      Benzodiazepines: pharmacology / Cognitive Dysfunction:
                      diagnosis / Creutzfeldt-Jakob Syndrome: diagnosis /
                      Diagnosis, Differential / Electroencephalography: methods /
                      Female / Humans / Male / Retrospective Studies / Status
                      Epilepticus: diagnosis / Status Epilepticus: drug therapy /
                      Benzodiazepines (NLM Chemicals)},
      cin          = {AG Gasser},
      ddc          = {610},
      cid          = {I:(DE-2719)1210000},
      pnm          = {345 - Population Studies and Genetics (POF3-345)},
      pid          = {G:(DE-HGF)POF3-345},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:29125945},
      doi          = {10.1016/j.seizure.2017.10.023},
      url          = {https://pub.dzne.de/record/139709},
}