Summary: A patient with biopsy-confirmed Creutzfeldt-Jakob disease (CJD) presented with unusual clinical features and electroencephalographic (EEG) findings. She had prominent aphasia and a left temporal slow-wave focus with epileptiform activity seen on EEG. Generalized dementia more typical for CJD evolved during a few weeks. We present unique documentation of her evolving clinical features and serial EEGs, which changed from a left temporal focus to periodic lateralizing epileptiform discharges, and ultimately to the typical bilateral periodic EEG pattern of CJD.
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