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

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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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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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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Variant Creutzfeldt-Jakob disease: pathology, epidemiology, and public health implications.
 

Author(s): Clare R Trevitt, Pramil N Singh

Journal: Am. J. Clin. Nutr.. 2003 Sep;78(3 Suppl):651S-656S.

 

Prion diseases, or transmissible spongiform encephalopathies, include Creutzfeldt-Jakob disease (CJD) in humans and scrapie and bovine spongiform encephalopathy (BSE) in animals. These neurodegenerative diseases are invariably fatal and can be transmitted by inoculation or dietary ...

Last Updated: 25 Aug 2003

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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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