Psychiatria pre prax 1/2015

Prion dementia

Prion diseases are very rare cause of the syndrom of dementia, their incidence is 1–2 cases per 1 million populations per year, which means about 1 per mille incidence of Alzheimer‘s disease. But this is a serious disease with a long incubation period and life limiting prognosis. The psychiatrist may be a doctor of the first line, who comes into contact with the patient. Important for the first expression of the suspicion of the prion diseases is the knowledge of the clinical image and the differences from the more commonly occurring dementia. For clinical practice, it is also important to know the epidemiological measures related to prion diseases including the dispensarisation and mandatory reporting.

Keywords: prion diseases, dementia, myoclonus, Creutzfeldt-Jakob disease.