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In rare circumstances, prion diseases also can be transmitted by exposure to prion-contaminated tissues or other biological materials obtained from individuals with prion disease.<efsa>It could be transmitted by Five cases of atypical BSE were reported in cattle across the and five more reported by countries.
Kuru, a transmissible spongiform encephalopathy, is a disease of the nervous system that causes physiological and neurological effects which ultimately lead to death. It is characterized by progressive cerebellar ataxia , or loss of coordination and control over muscle movements.
The human prion disease variant Creutzfeldt–Jakob disease, however, is thought to be caused by a prion that typically infects cattle, causing bovine spongiform encephalopathy and is transmitted through infected meat. [82] All known prion diseases are untreatable and fatal. [9] [83] [84]
Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer.TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar diseases such as BSE (mad cow disease) in cattle, Creutzfeldt–Jakob disease (CJD) in humans, and scrapie in sheep. [2]
Mad cow disease: A cow with BSE: Specialty: Neurology, veterinary medicine: Symptoms: Abnormal behavior, trouble walking, weight loss, inability to move [1] Complications: Variant Creutzfeldt-Jakob disease (if BSE-infected beef is eaten by humans) Usual onset: 4–5 years after exposure [2] Types: Classic, atypical [1] Causes: A type of prion ...
Creutzfeldt-Jacob disease (CJD) and variant Creutzfeldt-Jacob disease (vCJD), which is a form of mad cow disease, are prion diseases found in people. Mad cow disease is an example of a prion ...
The name "Creutzfeldt–Jakob disease" was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. [7] CJD is caused by abnormal folding of a protein known as a prion. [8] Infectious prions are misfolded proteins that can cause normally folded proteins to also become misfolded. [4]
Prion diseases, also called transmissible spongiform encephalopathies (TSEs), are neurodegenerative diseases of the brain thought to be caused by a protein that converts to an abnormal form called a prion. [16] [17] GSS is a very rare TSE, making its genetic origin nearly impossible to determine.