Variant Creutzfeldt-Jakob disease

Common Name(s)

Variant Creutzfeldt-Jakob disease

There are several known variants of Creutzfeldt-Jakob disease (CJD). These variants differ somewhat in the symptoms and course of the disease. For example, a variant form of the disease-called new variant or variant (nv-CJD, v-CJD), described in Great Britain and France, begins primarily with psychiatric symptoms, and has a longer than usual duration from onset of symptoms to death.  New variant CJD accounts for less than 1% of cases, and tends to affect younger people. It can result when someone is exposed to contaminated products. While classic CJD is not related to mad cow disease, new variant CJD (nvCJD) is an infectious form that is related to mad cow disease. The infection responsible for the disease in cows (bovine spongiform encephalitis) is believed to be the same one responsible for vCJD in humans. There have not been any cases of nvCJD reported in the U.S.

Another variant, called the panencephalopathic form, occurs primarily in Japan and has a relatively long course, with symptoms often progressing for several years. Scientists are trying to gain a better understanding about what causes these variations in the symptoms and course of the disease.
 

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

Population screening for variant Creutzfeldt-Jakob disease using a novel blood test: diagnostic accuracy and feasibility study.
 

Author(s): Graham S Jackson, Jesse Burk-Rafel, Julie Ann Edgeworth, Anita Sicilia, Sabah Abdilahi, Justine Korteweg, Jonathan Mackey, Claire Thomas, Guosu Wang, Jonathan M Schott, Catherine Mummery, Patrick F Chinnery, Simon Mead, John Collinge

Journal: JAMA Neurol. 2014 Apr;71(4):421-8.

 

Our study indicates a prototype blood-based variant Creutzfeldt-Jakob disease (vCJD) assay has sufficient sensitivity and specificity to justify a large study comparing vCJD prevalence in the United Kingdom with a bovine spongiform encephalopathy-unexposed population. In a clinical ...

Last Updated: 16 Apr 2014

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Postmortem findings in a case of variant Creutzfeldt-Jakob disease treated with intraventricular pentosan polysulfate.
 

Author(s): P K Newman, N V Todd, D Scoones, S Mead, R S G Knight, R G Will, J W Ironside

Journal: J. Neurol. Neurosurg. Psychiatr.. 2014 Aug;85(8):921-4.

 

A small number of patients with variant Creutzfeldt-Jakob disease (vCJD) have been treated with intraventicular pentosan polysulfate (iPPS) and extended survival has been reported in some cases. To date, there have been no reports on the findings of postmortem examination of the brain ...

Last Updated: 10 Jul 2014

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Variant Creutzfeldt-Jakob disease with extremely low lymphoreticular deposition of prion protein.
 

Author(s): Simon Mead, Jonathan D F Wadsworth, Marie-Claire Porter, Jacqueline M Linehan, Wojciech Pietkiewicz, Graham S Jackson, Sebastian Brandner, John Collinge

Journal: JAMA Neurol. 2014 Mar;71(3):340-3.

 

Human transmission of bovine spongiform encephalopathy causes the fatal neurodegenerative condition variant Creutzfeldt-Jakob disease (vCJD) and, based on recent human prevalence studies, significant subclinical prion infection of the UK population. To date, all clinical cases have ...

Last Updated: 11 Mar 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 11 free, full-text review articles on human participants. First 3 results:

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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Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: how safe is eating beef?
 

Author(s): Andres A Roma, Richard A Prayson

Journal: Cleve Clin J Med. 2005 Mar;72(3):185-6, 189-90, 192-4.

 

Cases of bovine spongiform encephalopathy (BSE, mad cow disease) have been found in North American cattle. Its human counterpart, called variant Creutzfeldt-Jakob disease (variant CJD), is rare but seems to be linked to eating diseased beef. Many questions remain about these diseases, ...

Last Updated: 13 Apr 2005

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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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