Refractory nonconvulsive status epilepticus in Creutzfeldt-Jakob disease.
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].
Aiguabella
Maria
M
Neurology Department, Hospital de Bellvitge, Hospitalet de Llobregat, Barcelona, Spain. 37785mam@comb.cat
Falip
Mercè
M
Veciana
Misericordia
M
Bruna
Jordi
J
Palasí
Antoni
A
Corral
Luisa
L
Herrero
Jose-Ignacio
JI
Boluda
Susana
S
Mora
Jaume
J
Iranzo
Alex
A
Serrano
Carme
C
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