Related dementia: Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease, often referred to as prion disease, is a rare, rapidly developing form of dementia caused by infectious proteins called prions. Prions are proteins that occur naturally in the brain and are normally harmless. However, prions cause disease when they take on a misshapen form, accumulate in the brain and kill off brain cells. Prion diseases affect both humans and animals.