Creutzfeldt–Jakob Disease

CJD is a rapidly progressive prion disease characterized by spongiform neurodegeneration and accumulation of misfolded prion protein. Clinical presentation includes rapidly progressive dementia, myoclonus, ataxia, and visual disturbances. Pathophysiology involves transmissible, pathogenic prions inducing neurodegeneration without inflammatory response. Diagnosis is based on clinical features, EEG showing periodic sharp wave complexes, MRI findings, cerebrospinal fluid biomarkers (14-3-3 protein), and definitive postmortem histopathology. Management is supportive, with no curative therapy; prognosis is poor, with median survival of approximately one year from onset.