Creutzfeldt-Jakob Disease

Common Name(s)

Creutzfeldt-Jakob Disease, CJD

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.

CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).

 

Advocacy and Support Organizations

 

Condition Specific Organizations

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

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The CJD Foundation

The Creutzfeldt-Jakob Disease Foundation consists of members who are concerned about the complexity of issues surrounding this fatal brain disease. Our mission is to support families and loved ones touched by CJD.

Last Updated: 11 Mar 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

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

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The CJD Foundation

The Creutzfeldt-Jakob Disease Foundation consists of members who are concerned about the complexity of issues surrounding this fatal brain disease. Our mission is to support families and loved ones touched by CJD.

http://www.cjdfoundation.org

Last Updated: 11 Mar 2013

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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 "Creutzfeldt-Jakob Disease" returned 498 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 "Creutzfeldt-Jakob Disease" returned 42 free, full-text review articles on human participants. First 3 results:

Management of notifications of donors with Creutzfeldt-Jakob disease (post-donation information).
 

Author(s): Gabriele Calizzani, Stefania Vaglio, Vito Vetrugno, Marisa Delbò, Luca Pani, Giuliano Grazzini

Journal: Blood Transfus. 2014 Jan;12(1):22-7.

 

Last Updated: 19 Feb 2014

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Creutzfeldt-Jakob disease: a case report and differential diagnoses.
 

Author(s): Gotaro Kojima, Brent K Tatsuno, Michiko Inaba, Stephanie Velligas, Kamal Masaki, Kore K Liow

Journal: Hawaii J Med Public Health. 2013 Apr;72(4):136-9.

 

Sporadic Creutzfeldt-Jakob disease is a rare neurodegenerative disorder of unknown etiology that causes rapidly progressive dementia. This disease is uniformly fatal and most patients die within 12 months. Clinical findings include myoclonus, visual disturbances, and cerebellar and ...

Last Updated: 24 Jun 2013

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Sensitivity to biases of case-control studies on medical procedures, particularly surgery and blood transfusion, and risk of Creutzfeldt-Jakob disease.
 

Author(s): Jesús de Pedro Cuesta, María Ruiz Tovar, Hester Ward, Miguel Calero, Andrew Smith, Concepción Alonso Verduras, Maurizio Pocchiari, Marc L Turner, Frode Forland, Daniel Palm, Robert G Will

Journal: Neuroepidemiology. 2012 ;39(1):1-18.

 

Evidence of risk of Creutzfeldt-Jakob disease (CJD) associated with medical procedures, including surgery and blood transfusion, is limited by susceptibility to bias in epidemiological studies.

Last Updated: 26 Jul 2012

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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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