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

Variant Creutzfeldt-Jakob Disease in a Patient with Heterozygosity at PRNP Codon 129.
 

Author(s): Tzehow Mok, Zane Jaunmuktane, Susan Joiner, Tracy Campbell, Catherine Morgan, Benjamin Wakerley, Farhad Golestani, Peter Rudge, Simon Mead, H Rolf Jäger, Jonathan D F Wadsworth, Sebastian Brandner, John Collinge

Journal: N. Engl. J. Med.. 2017 01;376(3):292-294.

 

Last Updated: 18 Jan 2017

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Sporadic Creutzfeldt-Jakob disease with unusual initial presentation as posterior reversible encephalopathy syndrome: a case report.
 

Author(s): Edgaras Dirzius, Renata Balnyte, Vesta Steibliene, Rymante Gleizniene, Inga Gudinaviciene, Andrius Radziunas, Kestutis Petrikonis

Journal:

 

Creutzfeldt - Jakob disease (CJD) is a rapidly progressive and fatal neurodegenerative prion disease. MRI findings are included in diagnostic criteria for probable CJD, giving a sensitivity and specificity more than 90%, but the atypical radiological presentations in the early stage ...

Last Updated: 23 Nov 2016

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Prion-Seeding Activity Is widely Distributed in Tissues of Sporadic Creutzfeldt-Jakob Disease Patients.
 

Author(s): Hanae Takatsuki, Takayuki Fuse, Takehiro Nakagaki, Tsuyoshi Mori, Ban Mihara, Masaki Takao, Yasushi Iwasaki, Mari Yoshida, Shigeo Murayama, Ryuichiro Atarashi, Noriyuki Nishida, Katsuya Satoh

Journal: EBioMedicine. 2016 Oct;12():150-155.

 

Human prion diseases are neurodegenerative disorders caused by abnormally folded prion proteins in the central nervous system. These proteins can be detected using the quaking-induced conversion assay. Compared with other bioassays, this assay is extremely sensitive and was used in ...

Last Updated: 10 Sep 2016

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

Systematic reviews in paediatric multiple sclerosis and Creutzfeldt-Jakob disease exemplify shortcomings in methods used to evaluate therapies in rare conditions.
 

Author(s): Steffen Unkel, Christian Röver, Nigel Stallard, Norbert Benda, Martin Posch, Sarah Zohar, Tim Friede

Journal:

 

Randomized controlled trials (RCTs) are the gold standard design of clinical research to assess interventions. However, RCTs cannot always be applied for practical or ethical reasons. To investigate the current practices in rare diseases, we review evaluations of therapeutic interventions ...

Last Updated: 22 Feb 2016

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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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Clinical Trial Information This information is provided by ClinicalTrials.gov

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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Genetic Characterization of Movement Disorders and Dementias
 

Status: Recruiting

Condition Summary: Ataxia; Dystonia; Parkinson's Disease; Amyotrophic Lateral Sclerosis; Corticobasal Degeneration; Multiple System Atrophy; Alzheimer's Disease; Lewy Body Dementia; Parkinson Disease-Dementia; Dentatorubral-pallidoluysian Atrophy; Creutzfeldt-Jakob Disease and Fatal Familial Insomnia; Fragile X-associated Tremor/Ataxia Syndrome; Krabbe's Disease; Niemann-Pick Disease, Type C; Neuronal Ceroid Lipofuscinosis

 

Last Updated: 30 Jun 2017

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