Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease

Epileptic Disorders : International Epilepsy Journal with Videotape
Maria AiguabellaCarme Serrano

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. [Published with video sequences].

Citations

May 9, 2013·Case Reports in Neurological Medicine·Harm J van der HornWouter J Schuiling
Apr 22, 2020·The International Journal of Neuroscience·Grammatiki KatsikakiSevasti Bostantjopoulou
Nov 13, 2015·Expert Review of Neurotherapeutics·José L Fernández-TorreMiguel A Hernández-Hernández
Nov 3, 2020·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Alicia Gonzalez-MartinezJosé Vivancos
Dec 15, 2020·Epilepsy & Behavior : E&B·Gashirai K MbizvoAndrew J Larner

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