<abstract xmlns="http://www.w3.org/1999/xhtml"><sec id="j_joepi-2017-0001_s_001"><h3>Introduction</h3><p>The differentiation between the clinical and electroencephalographic changes in noncon-vulsive status epilepticus (NCSE) and those in sporadic Creutzfeldt-Jakob disease (sCJD) is a crucial question.</p></sec><sec id="j_joepi-2017-0001_s_002"><h3>Case report</h3><p>A 77-year old woman was admitted because of fluctuating behavioural chancaseges, adynamia and apraxia since several months for diagnostic. The diagnosis of sCJD was suggested. Subsequently, she had a generalized tonic clonic seizure (GTCS) and the EEG revealed periodic lateralized epileptiform discharges and NCSE was considered.</p></sec><sec id="j_joepi-2017-0001_s_003"><h3>Discussion</h3><p>The presented case illustrates the dilemma in the differential diagnosis of sCJD and (symptomatic) NCSE in the light of the recently published new Salzburg consensus criteria and unified EEG terminology. Concerning these criteria, the patient showed after an initial generalized seizure and substantial clinical improvement after administration of antiepileptic drugs, persisting epileptic discharges and only subtle clinical ictal phenomena during the EEG patterns with typical spatiotemporal evolution as a correlate of a symptomatic NCSE. During the further course of the disease in the presented patient the picture changed into an encephalopathic pattern.</p></sec><sec id="j_joepi-2017-0001_s_004"><h3>Conclusion</h3><p>EEG criteria for the diagnosis of NCSE are complex. In our case the EEG resembled the pattern of NCSE in the postictal phase of a GTCS according to a classification of NCSE in use at this time. After initial responsiveness to antiepileptic medication the patient lost responsiveness to therapy displaying the typical encephalopathic EEG findings in sCJD. These findings may support the hypothesis of initial NCSE and transformation into prion protein induced encephalopathic EEG and demonstrated clinical usefulness of the Salzburg consensus criteria for NCSE.</p></sec></abstract>

IntroductionThe differentiation between the clinical and electroencephalographic changes in noncon-vulsive status epilepticus (NCSE) and those in sporadic Creutzfeldt-Jakob disease (sCJD) is a crucial question.Case reportA 77-year old woman was admitted because of fluctuating behavioural chancaseges, adynamia and apraxia since several months for diagnostic. The diagnosis of sCJD was suggested. Subsequently, she had a generalized tonic clonic seizure (GTCS) and the EEG revealed periodic lateralized epileptiform discharges and NCSE was considered.DiscussionThe presented case illustrates the dilemma in the differential diagnosis of sCJD and (symptomatic) NCSE in the light of the recently published new Salzburg consensus criteria and unified EEG terminology. Concerning these criteria, the patient showed after an initial generalized seizure and substantial clinical improvement after administration of antiepileptic drugs, persisting epileptic discharges and only subtle clinical ictal phenomena during the EEG patterns with typical spatiotemporal evolution as a correlate of a symptomatic NCSE. During the further course of the disease in the presented patient the picture changed into an encephalopathic pattern.ConclusionEEG criteria for the diagnosis of NCSE are complex. In our case the EEG resembled the pattern of NCSE in the postictal phase of a GTCS according to a classification of NCSE in use at this time. After initial responsiveness to antiepileptic medication the patient lost responsiveness to therapy displaying the typical encephalopathic EEG findings in sCJD. These findings may support the hypothesis of initial NCSE and transformation into prion protein induced encephalopathic EEG and demonstrated clinical usefulness of the Salzburg consensus criteria for NCSE.

The diagnostic dilemma of non-convulsive status epilepticus in sporadic Creutzfeldt-Jakob disease

Journal of Epileptology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

IntroductionThe differentiation between the clinical and electroencephalographic changes in noncon-vulsive status epilepticus (NCSE) and those in sporadic...

The diagnostic dilemma of non-convulsive status epilepticus in sporadic Creutzfeldt-Jakob disease

Publié en ligne: 05 juin 2017

Pages: 53 - 58

Reçu: 15 mars 2017

Accepté: 05 juin 2017

DOI: https://doi.org/10.21307/joepi-2017-0001

Mots clés
sporadic Creutzfeldt-Jakob disease, non-convulsive status epilepticus, EEG

© 2015 Matthias Wittstock et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

The diagnostic dilemma of non-convulsive status epilepticus in sporadic Creutzfeldt-Jakob disease

Publié en ligne: 05 juin 2017

Pages: 53 - 58

Reçu: 15 mars 2017

Accepté: 05 juin 2017

DOI: https://doi.org/10.21307/joepi-2017-0001

Mots cléssporadic Creutzfeldt-Jakob disease, non-convulsive status epilepticus, EEG

© 2015 Matthias Wittstock et al., published by Sciendo

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Mots clés
sporadic Creutzfeldt-Jakob disease, non-convulsive status epilepticus, EEG