What's variant Creutzfeldt-Jakob disease (vCJD)?
Variant Creutzfeldt-Jakob disease (vCJD) is a rare and fatal human neurodegenerative condition. As with Creutzfeldt-Jakob disease, vCJD is classified as a Transmissible Spongiform Encephalopathy (TSE) because of characteristic
spongy degeneration of the brain and its ability to be transmitted. vCJD is a new disease that was first described in March 1996.
Before the identification of vCJD, CJD was recognized to exist in only three forms. Sporadic cases, which have an unknown cause and occur throughout the world at the rate of about one per million people, account for 85–90% of CJD cases. Familial cases are associated with a gene mutation and make up 5–10% of all CJD cases. Iatrogenic cases result from the accidental transmission of the causative agent via contaminated surgical equipment or as a result of cornea or dura mater transplants or the administration of human-derived pituitary growth hormones. Less than 5% of CJD cases are iatrogenic.
In contrast to the traditional forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure, probably through food, to a TSE of cattle called Bovine Spongiform Encephalopathy (BSE).
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.