Variant Creutzfeldt-Jakob disease

Disease Overview

Variant Creutzfeldt-Jakob disease (vCJD) is a type of Creutzfeldt-Jakob disease (CJD) characterized by early psychiatric symptoms and cognitive decline. All forms of CJD belong to a rare family of progressive neurodegenerative disorders that affect both humans and animals, called prion diseases. The term “prion” refers to abnormal proteins within the brain, called prion proteins. vCJD, described primarily in the United Kingdom and France, accounts for less than 1% of cases of CJD, and tends to affect younger people. It can result when someone is exposed to contaminated products. The infection responsible for the disease in cows (bovine spongiform encephalitis) is believed to be the same one responsible for vCJD in humans.[928][929][930]

Another variant, called the panencephalopathic form, occurs primarily in Japan and has a relatively long course, with symptoms often progressing for several years. Scientists are trying to gain a better understanding about what causes these variations in the symptoms and course of the disease.[928]There is no specific treatment for CJD or vCJD, so the goal is to make a person as comfortable as possible.[928][929][930]

Synonyms

• Variant Creutzfeldt-Jacob disease
• VCJD
• Variant CJD
• New variant of CJD
• Nv-CJD