Creutzfeldt Jakob disease

The Creutzfeldt–Jakob disease - A rare, progressive degenerative disease of the brain. Creutzfeldt–Jakob disease (CJD) is thought to be caused by infection with a prion (a type of infectious protein). A similar agent causes scrapie in sheep and bovine spongiform encephalopathy (BSE) in cattle.

TYPES:
One form of CJD largely affects middleaged or elderly people, and appears to have no obvious cause. A second form occurs in younger people. and this is associated with contamination during brain surgery or transplants from infected people, or with treatment using infected human growth hormone or gonadotrophins.

A third form, new variant (nv) CJD, was first identified in 1995 and affects people in their teens and 20s. It is thought to be acquired by eating beef infected with BSE. This form causes pathological changes in the brain that are similar to the changes found in cattle suffering from BSE.

SYMPTOMS:
Symptoms are broadly similar for all forms of the disease. Slowly progressive dementia (deterioration in brain function) and myoclonus (sudden muscular contractions) occur; coordination diminishes; the intellect and personality deteriorate; and blindness may develop. As the disease progresses, speech is lost and the body becomes rigid.

OUTLOOK:
There is no treatment, and death usually occurs within two to three years.