Creutzfeldt-Jakob disease

Common Name(s)

Creutzfeldt-Jakob disease, CJD

Creutzfeldt-Jakob disease (CJD) is a type of rapidly progressive brain disorder known as prion disease. Prion disease is very rare and affects about one in every one million people per year. Symptoms of CJD typically develop around age 60 and can include trouble with memory along with changes in behavior, vision, and coordination. As the disease progresses, the severity of the symptoms increase.

The three variations of CJD are labeled as sporadic, hereditary, and acquired. Researchers believe the sporadic variation, which is most common, could be due to an incorrectly folded protein in the body, called an infectious prion. The prions clump together and cause damage to the brain, leading to the symptoms of CJD. The hereditary variation, which occurs much less often, is also caused by clumped prions. However, in the hereditary version, the abnormally folded prions are caused by a change (mutation) in the PRNP gene. The acquired variation of CJD is the least common and can occur when an individual comes in contact with the brain tissue or spinal fluid of an affective organism, such as eating the brain tissue of an affected cow (also known as mad cow disease). This variation accounts for about 1% of CJD cases.

Because CJD has symptoms similar to other forms of dementia, a doctor may attempt to rule out other conditions before suggesting a diagnosis of CJD. A medical professional may also perform a brain scan, such as an MRI, to look for changes in the brain tissue that are characteristic of CJD. The current treatments available for CJD focus on minimizing the symptoms associated with the disease. Researchers are trying to better understand what causes CJD in order to develop more effective treatments. If you or a family member has been diagnosed with CJD, talk with your doctor and specialists about the most current treatment options. Support groups are a good resource for additional information and support.

Source: Advocacy organizations associated with the condition.

 

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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General Support Organizations

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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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General Support Organizations

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

A genome wide association study links glutamate receptor pathway to sporadic Creutzfeldt-Jakob disease risk.
 

Author(s): Pascual Sanchez-Juan, Matthew T Bishop, Gabor G Kovacs, Miguel Calero, Yurii S Aulchenko, Anna Ladogana, Alison Boyd, Victoria Lewis, Claudia Ponto, Olga Calero, Anna Poleggi, Ángel Carracedo, Sven J van der Lee, Thomas Ströbel, Fernando Rivadeneira, Albert Hofman, Stéphane Haïk, Onofre Combarros, José Berciano, Andre G Uitterlinden, Steven J Collins, Herbert Budka, Jean-Philippe Brandel, Jean Louis Laplanche, Maurizio Pocchiari, Inga Zerr, Richard S G Knight, Robert G Will, Cornelia M van Duijn

Journal:

 

We performed a genome-wide association (GWA) study in 434 sporadic Creutzfeldt-Jakob disease (sCJD) patients and 1939 controls from the United Kingdom, Germany and The Netherlands. The findings were replicated in an independent sample of 1109 sCJD and 2264 controls provided by a multinational ...

Last Updated: 29 Apr 2015

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Recent US Case of Variant Creutzfeldt-Jakob Disease-Global Implications.
 

Author(s): Atul Maheshwari, Michael Fischer, Pierluigi Gambetti, Alicia Parker, Aarthi Ram, Claudio Soto, Luis Concha-Marambio, Yvonne Cohen, Ermias D Belay, Ryan A Maddox, Simon Mead, Clay Goodman, Joseph S Kass, Lawrence B Schonberger, Haitham M Hussein

Journal: Emerging Infect. Dis.. 2015 May;21(5):750-9.

 

Variant Creutzfeldt-Jakob disease (vCJD) is a rare, fatal prion disease resulting from transmission to humans of the infectious agent of bovine spongiform encephalopathy. We describe the clinical presentation of a recent case of vCJD in the United States and provide an update on diagnostic ...

Last Updated: 22 Apr 2015

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MRI detection of prion protein plaques in variant Creutzfeldt-Jakob disease.
 

Author(s): Harpreet Hyare, Po-Wah So, Sebastian Brandner, John Collinge, Harold G Parkes

Journal: Neurology. 2015 Apr;84(14):1498-9.

 

Last Updated: 7 Apr 2015

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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 47 free, full-text review articles on human participants. First 3 results:

Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems.
 

Author(s): Atsushi Kobayashi, Yuichi Matsuura, Shirou Mohri, Tetsuyuki Kitamoto

Journal:

 

Dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) can be divided into two subgroups that exhibit distinct clinical and neuropathological features, with the majority represented by a non-plaque-type of dCJD (np-dCJD) and the minority by a plaque-type of dCJD (p-dCJD). The ...

Last Updated: 8 Apr 2014

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Interpretation of cerebrospinal fluid protein tests in the diagnosis of sporadic Creutzfeldt-Jakob disease: an evidence-based approach.
 

Author(s): Michael B Coulthart, Gerard H Jansen, Neil R Cashman

Journal: CMAJ. 2014 Jun;186(9):E333-9.

 

Last Updated: 10 Jun 2014

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Creutzfeldt-Jakob disease: report of four cases and review of the literature.
 

Author(s): Fatma Öz Atalay, Şahsine Tolunay, Gonca Özgün, Ahmet Bekar, Mehmet Zarifoğlu

Journal: Turk Patoloji Derg. 2015 ;31(2):148-52.

 

Creutzfeldt-Jakob disease is a very rare, progressive neurodegenerative disorder that is incurable and always fatal. It is one of the transmissible spongiform encephalopathies caused by prions. Multiple vacuoles in neuropil and neuronal loss in the gray matter gives the classical ...

Last Updated: 6 May 2015

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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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The Role of the Coagulation Pathway at the Synapse in Prion Diseases
 

Status: Not yet recruiting

Condition Summary: Creutzfeldt-Jakob Syndrome

 

Last Updated: 23 Jun 2015

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