When Dementia Evolves Too Fast: Recognizing Sporadic Prion Disease

Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins. This series describes four cases of probable sporadic Creutzfeldt–Jakob disease (sCJD), which are geographically distinct. Patients presented with prominent behavioral and personality changes, lack of response to psychotropics, followed by ataxia, akinesia with myoclonus, and global rigidity. Magnetic resonance imaging consistently showed cortical ribboning, and cerebrospinal fluid 14-3-3 protein was positive. The resemblance of sporadic CJD to common psychiatric and neurological conditions often leads to missed or late diagnosis.