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

Similarities of Variant Creutzfeldt-Jakob Disease Strain in Mother and Son in Spain to UK Reference Case.
 

Author(s): Abigail B Diack, Aileen Boyle, Diane Ritchie, Chris Plinston, Dorothy Kisielewski, Jesús de Pedro-Cuesta, Alberto Rábano, Robert G Will, Jean C Manson

Journal: Emerging Infect. Dis.. 2017 09;23(9):1593-1596.

 

We investigated transmission characteristics of variant Creutzfeldt-Jakob disease in a mother and son from Spain. Despite differences in patient age and disease manifestations, we found the same strain properties in these patients as in UK vCJD cases. A single strain of agent appears ...

Last Updated: 31 Dec 1969

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Protective Effect of Val-PrP against Bovine Spongiform Encephalopathy but not Variant Creutzfeldt-Jakob Disease.
 

Author(s): Natalia Fernández-Borges, Juan Carlos Espinosa, Alba Marín-Moreno, Patricia Aguilar-Calvo, Emmanuel A Asante, Tetsuyuki Kitamoto, Shirou Mohri, Olivier Andréoletti, Juan María Torres

Journal: Emerging Infect. Dis.. 2017 09;23(9):1522-1530.

 

Bovine spongiform encephalopathy (BSE) is the only known zoonotic prion that causes variant Creutzfeldt-Jakob disease (vCJD) in humans. The major risk determinant for this disease is the polymorphic codon 129 of the human prion protein (Hu-PrP), where either methionine (Met) or valine ...

Last Updated: 31 Dec 1969

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Distribution and Quantitative Estimates of Variant Creutzfeldt-Jakob Disease Prions in Tissues of Clinical and Asymptomatic Patients.
 

Author(s): Jean Y Douet, Caroline Lacroux, Naima Aron, Mark W Head, Séverine Lugan, Cécile Tillier, Alvina Huor, Hervé Cassard, Mark Arnold, Vincent Beringue, James W Ironside, Olivier Andréoletti

Journal: Emerging Infect. Dis.. 2017 06;23(6):946-956.

 

In the United-Kingdom, ≈1 of 2,000 persons could be infected with variant Creutzfeldt-Jakob disease (vCJD). Therefore, risk of transmission of vCJD by medical procedures remains a major concern for public health authorities. In this study, we used in vitro amplification of prions ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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Variant Creutzfeldt-Jakob disease: an update.
 

Author(s): James W Ironside

Journal: Folia Neuropathol. 2012 ;50(1):50-6.

 

Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease caused by the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, with smaller numbers in 11 other countries. All definite vCJD cases have occurred in methionine homozygotes at codon 129 ...

Last Updated: 31 Dec 1969

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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: 31 Dec 1969

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