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.

Source: Advocacy organizations associated with the condition.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

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 112 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

Go To URL
Rapid and Highly Sensitive Detection of Variant Creutzfeldt-Jakob Disease Abnormal Prion Protein on Steel Surfaces by Protein Misfolding Cyclic Amplification: Application to Prion Decontamination Studies.
 

Author(s): Maxime Belondrade, Simon Nicot, Vincent Béringue, Joliette Coste, Sylvain Lehmann, Daisy Bougard

Journal:

 

The prevalence of variant Creutzfeldt-Jakob disease (vCJD) in the population remains uncertain, although it has been estimated that 1 in 2000 people in the United Kingdom are positive for abnormal prion protein (PrPTSE) by a recent survey of archived appendix tissues. The prominent ...

Last Updated: 23 Jan 2016

Go To URL
Revisiting the Heidenhain Variant of Creutzfeldt-Jakob Disease: Evidence for Prion Type Variability Influencing Clinical Course and Laboratory Findings.
 

Author(s): Simone Baiardi, Sabina Capellari, Anna Ladogana, Silvia Strumia, Mario Santangelo, Maurizio Pocchiari, Piero Parchi

Journal: J. Alzheimers Dis.. 2016 ;50(2):465-76.

 

The Heidenhain variant defines a peculiar clinical presentation of sporadic Creutzfeldt-Jakob disease (sCJD) characterized by isolated visual disturbances at disease onset and reflecting the early targeting of prions to the occipital cortex. Molecular and histopathological typing, ...

Last Updated: 3 Feb 2016

Go To URL

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 16 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

Go To URL
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: 16 Apr 2012

Go To URL
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

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

There are currently no related results available in GeneReviews.

There are currently no related results available in Genetic Testing Registry.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.