Abnormal prion proteins accumulate in the brain and the spinal cord, damaging neurones (see NEURON(E)) and producing small cavities. Diagnosis can be made by tonsil (see TONSILS) biopsy, although work is under way to develop a diagnostic blood test. Abnormal prion proteins are unusually resistant to inactivation by chemicals, heat, X-RAYS or ULTRAVIOLET RAYS (UVR). They are resistant to cellular degradation and can convert normal prion proteins into abnormal forms. Human prion diseases, along with scrapie in sheep and BSE in cattle, belong to a group of disorders known as transmissible spongiform encephalopathies. Abnormal prion proteins can transfer from one animal species to another, and variant CJD has occurred as a result of consumption of meat from cattle infected with BSE.
From 1995 to 1999, a scienti?c study of tonsils and appendixes removed at operation suggested that the prevalence of prion carriage may be as high as 120 per million. It is not known what percentage of these might go on to develop disease.
One precaution is that, since 2003, all surgical instruments used in brain biopsies have had to be quarantined and disposable instruments are now used in tonsillectomy.
Measures have also been introduced to reduce the risk of transmission of CJD from transfusion of blood products.
In the past, CJD has also been acquired from intramuscular injections of human cadaveric pituitary-derived growth hormone and corneal transplantation.
The most common form of CJD remains the sporadic variety, although the eventual incidence of variant CJD may not be known for many years.... creutzfeldt-jakob disease (cjd)
Wernicke’s encephalopathy is a degenerative condition of the brain caused by a deficiency of vitamin B1 (see Wernicke–Korsakoff syndrome).
Hepatic encephalopathy is caused by the effect on the brain of toxic substances (see toxin) that have built up in the blood as a result of liver failure. It may lead to impaired consciousness, memory loss, a change in personality tremors, and seizures.
Bovine spongiform encephalopathy, or , is a disorder contracted by cattle after they are given feed containing material from sheep or cattle.
The cause seems to be an infective agent known as a prion.
Some cases of new variant Creutzfeldt–Jakob disease in humans have been attributed to infection with the prions responsible for , probably transmitted in meat products.... encephalopathy
The disease is caused by a “slow” virus or prion, which has a long incubation period and is spread by cannibalism.
Symptoms include progressive difficulty in controlling movements and dementia.
The study of kuru led to the identification of prions, which also play a role in Creutzfeldt–Jakob disease and BSE.... kuru