title: Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease.
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].
authors.affiliation: Neurology Department, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Spain. 37785mam@comb.cat
authors.forename: Maria
authors.initials: M
authors.lastname: Aiguabella
authors.forename: Mercè
authors.initials: M
authors.lastname: Falip
authors.forename: Misericordia
authors.initials: M
authors.lastname: Veciana
authors.forename: Jordi
authors.initials: J
authors.lastname: Bruna
authors.forename: Antoni
authors.initials: A
authors.lastname: Palasí
authors.forename: Luisa
authors.initials: L
authors.lastname: Corral
authors.forename: Jose-Ignacio
authors.initials: JI
authors.lastname: Herrero
authors.forename: Susana
authors.initials: S
authors.lastname: Boluda
authors.forename: Jaume
authors.initials: J
authors.lastname: Mora
authors.forename: Alex
authors.initials: A
authors.lastname: Iranzo
authors.forename: Carme
authors.initials: C
authors.lastname: Serrano
mesh_terms: D000328:Adult; D002421:Caudate Nucleus / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D003342:Corpus Striatum / Q000473:pathology; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology / Q000503:physiopathology*; D004569:Electroencephalography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013226:Status Epilepticus / Q000209:etiology* / Q000473:pathology