Creutzfeldt-Jakob disease (CJD) is among very rare, progressive, untreatable, neurodegenerative prion diseases. While the incidence is reported as 1/1000000 years in European countries, sporadic cases are rarely presentedin Turkey. Clinical findings are in the form of rapid progressive dementia, myoclonus, cerebellar, pyramidal and extra pyramidal symptoms. Definitive diagnosis is established by histopathological examination. Our case is a 64-year-old male and 70-year-old female patients admitted with dizziness emerged as sub-acute, weakness, nausea, vomiting, insomnia, imbalance and additional neuropsychiatric complaints. Focal activity slowness and common periodic sharp wave activities were observed in EEG. CJD was considered because bilateral symmetrical diffusion limitation was observed at both sides in the basal ganglia level in cranial diffusion MRIs and 14-3-3 protein was resulted as positive in CSF examinations.CJD, which is a very rare disease in patients presenting with progressive neuropsychiatric symptoms and seizure, is one of the diagnosis to be considered and we wanted to emphasize that there is no treatment for itand preventive measures should be taken.
Creutzfeldt-Jakob disease; Dementia; Myoclonus