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Epileptic Disorders

, Volume 12, Issue 3, pp 239–242 | Cite as

Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease

  • Maria Aiguabella
  • Mercè Falip
  • Misericordia Veciana
  • Jordi Bruna
  • Antoni Palasí
  • Luisa Corral
  • Jose-Ignacio Herrero
  • Susana Boluda
  • Jaume Mora
  • Alex Iranzo
  • Carme Serrano
Clinical Commentary with Video Sequences

Abstract

Creutzfeldt-Jakob disease (CJD) is a rare human transmissible spongiform subacute encephalopathy. The most common clinical manifestations of CJD include rapidly progressive dementia, behavioural changes, cerebellar dysfunction and myoclonus. Other seizure types are rare and nonconvulsive status epilepticus (SE) is exceptional. We report a case of a 44-year-old man who presented a psychotic episode followed by akinetic mutism and refractory nonconvulsive SE. The final diagnosis was CJD. Continuous video-EEG monitoring revealed the ictal pattern of nonconvulsive SE to be periodic sharp wave complexes characteristic of CJD.

Key words

electroencephalography status epilepticus nonconvulsive Creutzfeldt-Jakob disease 

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Copyright information

© John Libbey Eurotext and Springer 2010

Authors and Affiliations

  • Maria Aiguabella
    • 1
    • 7
  • Mercè Falip
    • 1
  • Misericordia Veciana
    • 1
  • Jordi Bruna
    • 1
  • Antoni Palasí
    • 1
  • Luisa Corral
    • 2
  • Jose-Ignacio Herrero
    • 2
  • Susana Boluda
    • 3
  • Jaume Mora
    • 4
  • Alex Iranzo
    • 5
  • Carme Serrano
    • 6
  1. 1.Neurology Department, Hospital de BellvitgeHospitalet de LlobregatBarcelonaSpain
  2. 2.Intensive Care Unit, Hospital de BellvitgeHospitalet de LlobregatBarcelonaSpain
  3. 3.Anatomical Pathology Department, Hospital de BellvitgeHospitalet de LlobregatBarcelonaSpain
  4. 4.Nuclear Medicine Department, Hospital de BellvitgeHospitalet de LlobregatBarcelonaSpain
  5. 5.Neurology DepartmentHospital ClinicBarcelonaSpain
  6. 6.Neurology DepartmentHospital de Sant Joan de Deu de Martorell, MartorellBarcelonaSpain
  7. 7.Hospitalet de LlobregatHospital Universitari de Bellvitge, Servei de NeurologiaBarcelonaSpain

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