John Libbey Eurotext

Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease Volume 12, issue 3, September 2010

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  • Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease

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Authors
Neurology Department, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Intensive Care Unit, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Anatomical Pathology Department, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Nuclear Medicine Department, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Neurology Department, Hospital Clinic, Barcelona, Neurology Department, Hospital de Sant Joan de Deu de Martorell, Martorell, Barcelona, Spain

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]