| Variant Creutzfeldt–Jakob disease | |
|---|---|
| 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]
References edit
- ^ a b c d "Clinical and Pathologic Characteristics | Variant Creutzfeldt-Jakob Disease, Classic (CJD)". CDC. 10 February 2015. Archived from the original on 27 March 2019. Retrieved 22 January 2018.
- ^ a b c d e f "Classic CJD versus Variant CJD". CDC. 11 February 2015. Archived from the original on 27 March 2019. Retrieved 23 January 2018.
- ^ a b Ironside, JW (Jul 2010). "Variant Creutzfeldt–Jakob disease". Haemophilia. 16 Suppl 5: 175–80. doi:10.1111/j.1365-2516.2010.02317.x. PMID 20590878.
- ^ a b "Treatment Variant Creutzfeldt-Jakob Disease". CDC. 10 February 2015. Archived from the original on 27 March 2019. Retrieved 23 January 2018.
- ^ a b c d e f g Ironside, JW (2012). "Variant Creutzfeldt–Jakob disease: an update". Folia Neuropathologica. 50 (1): 50–6. PMID 22505363.
- ^ a b c "About vCJD". CDC. 10 February 2015. Archived from the original on 2 May 2019. Retrieved 22 January 2018.
- ^ Ferri, Fred F. (2017). Ferri's Clinical Advisor 2018 E-Book: 5 Books in 1. Elsevier Health Sciences. p. 343. ISBN 9780323529570. Archived from the original on 2020-02-04. Retrieved 2018-01-23.