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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General Support Organizations

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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 100 free, full-text research articles on human participants. First 3 results:

Development of dose-response models of Creutzfeldt-Jakob disease infection in nonhuman primates for assessing the risk of transfusion-transmitted variant Creutzfeldt-Jakob disease.
 

Author(s): Yin Huang, Luisa Gregori, Steven A Anderson, David M Asher, Hong Yang

Journal: J. Virol.. 2014 Dec;88(23):13732-6.

 

Estimates for the risk of transmitting variant Creutzfeldt-Jakob disease (vCJD) via blood transfusion have relied largely on data from rodent experiments, but the relationship between dose (amount of infected blood) and response (vCJD infection) has never been well quantified. The ...

Last Updated: 31 Oct 2014

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Prions in the urine of patients with variant Creutzfeldt-Jakob disease.
 

Author(s): Fabio Moda, Pierluigi Gambetti, Silvio Notari, Luis Concha-Marambio, Marcella Catania, Kyung-Won Park, Emanuela Maderna, Silvia Suardi, Stéphane Haïk, Jean-Philippe Brandel, James Ironside, Richard Knight, Fabrizio Tagliavini, Claudio Soto

Journal: N. Engl. J. Med.. 2014 Aug;371(6):530-9.

 

Prions, the infectious agents responsible for transmissible spongiform encephalopathies, consist mainly of the misfolded prion protein (PrP(Sc)). The unique mechanism of transmission and the appearance of a variant form of Creutzfeldt-Jakob disease, which has been linked to consumption ...

Last Updated: 7 Aug 2014

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Accelerated, spleen-based titration of variant Creutzfeldt-Jakob disease infectivity in transgenic mice expressing human prion protein with sensitivity comparable to that of survival time bioassay.
 

Author(s): Sophie Halliez, Fabienne Reine, Laetitia Herzog, Emilie Jaumain, Stéphane Haïk, Human Rezaei, Jean-Luc Vilotte, Hubert Laude, Vincent Béringue

Journal: J. Virol.. 2014 Aug;88(15):8678-86.

 

The dietary exposure of the human population to the prions responsible for the bovine spongiform encephalopathy (BSE) epizooty has led to the emergence of variant Creutzfeldt-Jakob disease (vCJD). This fatal, untreatable neurodegenerative disorder is a growing public health concern ...

Last Updated: 29 Jul 2014

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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 13 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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