Variant Creutzfeldt–Jakob disease
Degenerative brain disease caused by prions / From Wikipedia, the free encyclopedia
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Variant Creutzfeldt–Jakob disease (vCJD), commonly referred to as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family.[7] Initial symptoms include psychiatric problems, behavioral changes, and painful sensations.[1] In the later stages of the illness, patients may exhibit poor coordination, dementia and involuntary movements.[2] The length of time between exposure and the development of symptoms is unclear, but is believed to be years to decades.[3] Average life expectancy following the onset of symptoms is 13 months.[1]
Variant Creutzfeldt–Jakob disease | |
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Other names | New variant Creutzfeldt–Jakob disease (nvCJD), human mad cow disease |
Biopsy of the tonsil in variant CJD. Prion protein immunostaining. | |
Specialty | Infectious disease, Neurology |
Symptoms | Initial: Psychiatric problems, behavioral changes, painful sensations[1] Later: Poor coordination, dementia, hallucinations, involuntary movements[2] |
Usual onset | Years after initial exposure[3] |
Duration | ~13-month life expectancy after onset of symptoms[1] |
Causes | Prions |
Risk factors | Eating beef from cows with bovine spongiform encephalopathy[3][4] |
Diagnostic method | Suspected based on symptoms, confirmed by brain biopsy[3] |
Differential diagnosis | Multiple sclerosis, standard Creutzfeldt-Jakob disease |
Prevention | Not eating contaminated beef |
Treatment | Supportive care[5] |
Prognosis | Always fatal[6] |
Frequency | Fewer than 250 reported cases as of 2012[7] |
It is caused by prions, which are misfolded proteins.[8] Spread is believed to be primarily due to eating bovine spongiform encephalopathy (BSE) infected beef.[7][8] Infection is also believed to require a specific genetic susceptibility.[4][7] Spread may potentially also occur via blood products or contaminated surgical equipment.[9] Diagnosis is by brain biopsy but can be suspected based on certain other criteria.[3] It is different from typical Creutzfeldt–Jakob disease, though both are due to prions.[8]
Treatment for vCJD involves supportive care.[5] As of 2020, 178 cases of vCJD have been recorded in the United Kingdom,[10] due to a 1990s outbreak, and 50 cases in the rest of the world.[7] The disease has become less common since 2000.[7] The typical age of onset is less than 30 years old.[3] It was first identified in 1996 by the National CJD Surveillance Unit in Edinburgh, Scotland.[7]