Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease is an incurable, terminal, neurodegenerative disease belonging to the transmissible spongiform encephalopathy group of prion diseases. Misfolded prion proteins cause normal prion proteins in the brain to adopt the same abnormal shape in a self-perpetuating cycle, producing sponge-like tissue damage and neuronal death. Early signs include memory problems, behavioral changes, poor coordination and visual disturbances, progressing to dementia, involuntary movements, blindness, deafness, weakness and coma; about 70 percent of patients die within a year of diagnosis. Cases are sporadic (about 85 percent, cause unknown), familial (10 to 15 percent, inherited mutations) or acquired through contaminated medical procedures or tissue exposure; the disease was first described by German neurologist Hans Gerhard Creutzfeldt in 1920.