Background: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease. Common first symptoms are dementia, cerebellar ataxia, visual disturbance, and psychiatric symptoms. Seizure as the first symptom of CJD is a very rare finding.
Case presentation: We experienced an elderly woman who presented initially with status epilepticus following repeated partial seizures in the course of Alzheimer disease (AD) dementia. Anti-convulsive therapy had no effect. Autopsy revealed definite CJD with AD pathology.
Coclusions: This is the first reported CJD case presenting with status epilepticus in the course of AD dementia.
Keywords: Alzheimer disease; Creutzfeldt-Jakob disease; Epilepsy; Seizure; Status epilepticus.