Variant Creutzfeldt-Jakob disease

Common Name(s)

Variant Creutzfeldt-Jakob disease, Bovine spongiform encephalopathy (BSE), Mad cow disease

Mad cow disease, also known as bovine spongiform encephalopathy (BSE), is a deadly disease which affects the central nervous system of adult cattle. The central nervous system includes the brain and spinal cord. If a human eats any beef containing brain or spinal cord tissue from an infected cow, they may develop the human form of the disease called variant Creutzfeldt–Jakob disease (vCJD). There is no evidence that consuming muscle meat (ground beef, steaks, or roasts), milk, or milk products can cause vCJD. Three cases prior to 2011 in the United Kingdom were due to a blood transfusion.

The symptoms of vCJD may include depression, apathy, anxiety, painful sensory symptoms, loss of coordination, and dementia. vCJD has been fatal in all human cases. Diagnosis is made by examining the symptoms and the progression of the condition. MRI scans, biopsy of the tonsils and an electroencephalogram (special recording of brain waves or EEG) may also be used. vCJD can only be confirmed after death.

There have been just over 200 cases of vCJD worldwide, most in the United Kingdom before 2008. Since 2008, the United Kingdom has had less than 2 new cases per year. In order to control the spread of vCJD, many countries have regulations on their food supplies. For example, the United States removes high-risk parts of cows (such as the brain and spinal cord) from all beef products. China placed bans so that no cows from countries with known cases of the disease could enter.

The exact cause of vCJD is unknown, but researchers believe it may be caused by an improperly folded infectious protein called a prion. At present there is no cure for vCJD though research is ongoing. Medication may be used to slow the disease and help control certain symptoms. If you or a family member has been diagnosed with vCJD, talk to your doctor and specialists about the most current treatment options.

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Variant Creutzfeldt-Jakob disease" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Variant Creutzfeldt-Jakob disease" returned 106 free, full-text research articles on human participants. First 3 results:

Variants of PLCXD3 are not associated with variant or sporadic Creutzfeldt-Jakob disease in a large international study.
 

Author(s): Rubika Balendra, James Uphill, Claire Collinson, Ronald Druyeh, Gary Adamson, Holger Hummerich, Inga Zerr, Pierluigi Gambetti, John Collinge, Simon Mead

Journal:

 

Human prion diseases are relentlessly progressive neurodegenerative disorders which include sporadic Creutzfeldt-Jakob disease (sCJD) and variant CJD (vCJD). Aside from variants of the prion protein gene (PRNP) replicated association at genome-wide levels of significance has proven ...

Last Updated: 8 Apr 2016

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Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.
 

Author(s): Atul Maheshwari, Michael Fischer, Pierluigi Gambetti, Alicia Parker, Aarthi Ram, Claudio Soto, Luis Concha-Marambio, Yvonne Cohen, Ermias D Belay, Ryan A Maddox, Simon Mead, Clay Goodman, Joseph S Kass, Lawrence B Schonberger, Haitham M Hussein

Journal: Emerging Infect. Dis.. 2015 May;21(5):750-9.

 

Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic ...

Last Updated: 22 Apr 2015

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MRI detection of prion protein plaques in variant Creutzfeldt-Jakob disease.
 

Author(s): Harpreet Hyare, Po-Wah So, Sebastian Brandner, John Collinge, Harold G Parkes

Journal: Neurology. 2015 Apr;84(14):1498-9.

 

Last Updated: 7 Apr 2015

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Variant Creutzfeldt-Jakob disease" returned 14 free, full-text review articles on human participants. First 3 results:

The genetics of susceptibility to variant Creutzfeldt-Jakob disease.
 

Author(s): R Saba, S A Booth

Journal: Public Health Genomics. 2013 ;16(1-2):17-24.

 

The emergence of bovine spongiform encephalopathy (BSE) in cattle and, subsequently, its transmission to humans resulting in variant Creutzfeldt-Jakob disease (vCJD) in the UK has proved to be one of the major public health scares of the century. The oral route of infection, the long ...

Last Updated: 3 Apr 2013

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Review. The neuropathology of kuru and variant Creutzfeldt-Jakob disease.
 

Author(s): Catriona A McLean

Journal: Philos. Trans. R. Soc. Lond., B, Biol. Sci.. 2008 Nov;363(1510):3685-7.

 

A comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. Archival kuru and recent variant Creutzfeldt-Jakob disease (vCJD) cases reveal distinct lesional differences, particularly with respect to prion ...

Last Updated: 13 Oct 2008

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Insights into the management of emerging infections: regulating variant Creutzfeldt-Jakob disease transfusion risk in the UK and the US.
 

Author(s): Maya L Ponte

Journal: PLoS Med.. 2006 Oct;3(10):e342.

 

Variant Creutzfeldt-Jakob disease (vCJD) is a human prion disease caused by infection with the agent of bovine spongiform encephalopathy. After the recognition of vCJD in the UK in 1996, many nations implemented policies intended to reduce the hypothetical risk of transfusion transmission ...

Last Updated: 29 Nov 2006

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Notification of Donors With Positive Microbiology Markers
 

Status: Not yet recruiting

Condition Summary: Human Immunodeficiency Virus; Hepatitis B; Hepatitis C; Human T-lymphotropic Virus I & II; Creutzfeldt-Jakob Syndrome; HIV Infections

 

Last Updated: 12 Feb 2010

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