What is Creutzfeldt-Jakob disease?
Creutzfeldt-Jakob disease is degenerative brain disorder, known as a prion disease. Creutzfeldt-Jakob disease (CJD) is a rapidly progressive disease causing damage to the brain. It is one of a group of rare diseases that affects humans and animals, known as the transmissible spongiform encephalopathies or prion diseases. Creutzfeldt-Jakob disease is
characterised by dementia and walking difficulties. Death can occur up to two years after the first symptoms; however, most people die within six months. There is no treatment or cure.
Prion diseases affect both humans and certain animals, including cows and sheep. Creutzfeldt-Jakob Disease affects men and women between the ages of 50-75 years. The incident rate is reportedly one case per million people a year, although this may be a low estimate because the disease is very difficult to diagnose. There is no definitive diagnosis without a brain biopsy or an autopsy, and the procedures are not done very often because they do not help the patient. Mad cow disease (bovine spongiform encephalopathy) is a prion disease. Prion diseases are rare and fatal.
The prion that is believed to cause Creutzfeldt-Jakob exhibits an amino acid sequence and configuration which makes it insoluble in water, while the normal protein is highly soluble. Normal prion proteins are harmless, but infectious forms of the prion protein are thought to cause Creutzfeldt-Jakob disease and other diseases. As the numbers of defective prion proteins propagate and increase exponentially, the process leads to a huge load of insoluble prions in affected cells. This load of proteins disrupts cell function and causes cell death. Once the prion is transmitted, the defective proteins invade the brain like a forest fire and the patient dies within a few months (a few patients live for about 1-2 years). The defective protein can be transmitted by human growth hormone products, corneal grafts or dural grafts (acquired form) or it can be inherited (hereditary form) or appear for the first time in the patient (sporadic form). In the latter two forms the defective protein is not transmitted from an external source but already exists in the genes of the individual.
Cannibalism has also been implicated as a transmission mechanism for abnormal prions, the disease being known as Kuru found primarily among women and children of the Fore tribe in Papua New Guinea. The disease has also been shown to result from usage of HGH drawn from the pituitary glands of cadavers who died from Creutzfeldt-Jakob Disease, though the known incidence of this cause is (as of April 2004) quite small. Infection through HGH usage is restricted to patients in the U.S. who were treated with HGH during or before 1977, when newer methods of HGH purification were adopted.
More information on prion diseases (Creutzfeldt-Jakob disease, fatal familial insomnia, kuru)
What're prion diseases? - Prion diseases (transmissible spongiform encephalopathies) are a group of inherited and spontaneous neurodegenerative disorders affecting animals and humans.
What is Creutzfeldt-Jakob disease? - Creutzfeldt-Jakob disease is degenerative brain disorder. Creutzfeldt-Jakob disease (CJD) is a rapidly progressive disease causing damage to the brain.
What types of Creutzfeldt-Jakob disease are there? - There are three types of Creutzfeldt-Jakob Disease: sporadic (or classical), hereditary (or familial) and acquired (or iatrogenic).
What's variant Creutzfeldt-Jakob disease (vCJD)? - Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition. vCJD affects younger patients.
What causes Creutzfeldt-Jakob disease? - There are three main causes for classic CJD. Creutzfeldt-Jakob disease through infection is transmitted by prions.
What're the symptoms of Creutzfeldt-Jakob disease? - Symptoms of Creutzfeldt-Jakob disease include forgetfulness and nervousness, jerky, trembling hand movements, unsteady gait, myoclonus.
How is Creutzfeldt-Jakob disease diagnosed? - Creutzfeldt-Jakob disease is diagnosed by a clinical neurological exam and electroencephalography (EEG).
What's the treatment for Creutzfeldt-Jakob disease? - There is no cure for Creutzfeldt-Jakob disease, and no treatment which slows the progression of the disease.
How to prevent Creutzfeldt-Jakob disease? - Creutzfeldt-Jakob disease is prevented by handling their fluids and tissues with extreme caution and by using special sterilization methods to disinfect equipment.
What is fatal familial insomnia? - Fatal familial insomnia is caused by prions, similar to Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.
What is Kuru? - Kuru is a degenerative nerve disease caused by a prion (infectious protein) transmitted to humans via contaminated human brain tissue.