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 | Neurology |
Symptoms | Psychiatric problems, behavioral changes, painful sensations [1] |
Usual onset | Less than 30 years old [2] |
Causes | Prion |
Risk factors | Eating beef from animals with bovine spongiform encephalopathy [2] [3] |
Diagnostic method | Brain biopsy [2] |
Treatment | Supportive care [4] |
Prognosis | ~13-month life expectancy [1] |
Frequency | Fewer than 250 reported cases as of 2012 [5] |
Variant Creutzfeldt–Jakob disease (vCJD) is a type of brain disease within the transmissible spongiform encephalopathy family. [5] Symptoms include psychiatric problems, behavioral changes, and painful sensations. [1] The length of time between exposure and the development of symptoms is unclear, but is believed to be years. [2] Average life expectancy following the onset of symptoms is 13 months. [1]
It is caused by prions, which are mis-folded proteins. [6] Spread is believed to be primarily due to eating bovine spongiform encephalopathy (BSE)-infected beef. [5] [6] Infection is also believed to require a specific genetic susceptibility. [3] [5] Spread may potentially also occur via blood products or contaminated surgical equipment. [7] Diagnosis is by brain biopsy but can be suspected based on certain other criteria. [2] It is different from classic Creutzfeldt–Jakob disease, though both are due to prions. [6]
Treatment for vCJD involves supportive care. [4] As of 2012 about 170 cases of vCJD have been recorded in the United Kingdom and 50 cases in the rest of the world. [5] The disease has become less common since 2000. [5] The typical age of onset is less than 30 years old. [2] It was first identified in 1996 by the National CJD Surveillance Unit in Edinburgh, Scotland. [5]