Creutzfeldt-Jakob Disease Definition & Overview

Definition

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive dementia and neuromuscular problems.

There are four types of CJD:

• Sporadic (classical) CJD—most common type; usually affects people age 50 and older
• Familial CJD—an inherited form of the disease
• Iatrogenic CJD—contracted through medical procedures, such as injections of growth hormone or receipt of corneal transplants or dura mater implants from affected donors
• New variant CJD (nvCJD) —relatively new form of CJD. It is caused by eating contaminated beef products, which may cause bovine spongiform encephalopathy (BSE). BSE is commonly known as mad cow disease. nvCJD differs from other forms of CJD because it affects younger people and has a longer average time course.