Information about CJD

Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder that causes rapidly progressing dementia. The disease affects about one in one million people worldwide each year, including 250 to 300 Americans. CJD can be transmitted through infected tissue (usually through organ or tissue transplantation), can be inherited, or can occur with no known explanation. CJD typically strikes people over age 60 and leads to death in about a year.

A disorder with similar symptoms appeared in England in the mid-1990s, about 10 years after an outbreak of bovine spongiform encephalopathy (BSE, or mad cow disease) in cattle. BSE was linked to cattle feed that contained infected animal tissue. The human form of the disease, known as new variant CJD (vCJD), has been reported in about 150 people worldwide, most of whom have been under age 30. All are believed to have eaten tainted beef in Europe during the mad cow disease epidemic.

The disorder progresses more slowly than classic CJD (about 14 months) but inevitably leads to death. As of June 2, 2014, variant CJD cases have been reported from the following countries: 177 from the United Kingdom, 27 from France, 5 from Spain, 4 from Ireland, 4 from the United States, 3 in the Netherlands, 2 in Portugal, 2 in Italy, 2 in Canada and one each from Japan, Saudi Arabia, and Taiwan, according to the Centers for Disease Control and Prevention (CDC).

No treatments exist for classic CJD or vCJD. The number of cases of vCJD has plummeted since laws were passed banning any animal remains in cattle feed. If a family member dies of suspected CJD or vCJD, an autopsy should be performed to determine the precise cause of death.

Updated by Remedy Health Media

Publication Review By: Peter V. Rabins, M.D., M.P.H.

Published: 09 Mar 2011

Last Modified: 28 Oct 2014