Health Encyclopedia > Health Topics

Creutzfeldt Jakob Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Creutzfeldt Jakob Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • CJD
  • Jakob-Creutzfeldt Disease
  • Subacute Spongiform Encephalopathy
  • Jakob's Disease

Disorder Subdivisions

  • Variant Creutzfeldt-Jakob Disease (V-CJD)

General Discussion

Creutzfeldt-Jakob disease (CJD) is an extremely rare degenerative brain disorder (i.e., spongiform encephalopathy) characterized by sudden development of rapidly progressive neurological and neuromuscular symptoms. With symptom onset, affected individuals may develop confusion, depression, behavioral changes, impaired vision, and/or impaired coordination. As the disease progresses, there may be rapidly progressive deterioration of cognitive processes and memory (dementia), resulting in confusion and disorientation, impairment of memory control, personality disintegration, agitation, restlessness, and other symptoms and findings. Affected individuals also develop neuromuscular abnormalities such as muscle weakness and loss of muscle mass (wasting); irregular, rapid, shock-like muscle spasms (myoclonus); and/or relatively slow, involuntary, continual writhing movements (athetosis), particularly of the arms and legs. Later stages of the disease may include further loss of physical and intellectual functions, a state of unconsciousness (coma), and increased susceptibility to repeated infections of the respiratory tract (e.g., pneumonia). In many affected individuals, life-threatening complications may develop less than a year after the disorder becomes apparent.



In approximately 90 percent of cases, CJD appears to occur randomly for no apparent reason (sporadically). About 10 percent of affected individuals may have a hereditary predisposition for the disorder. Reports in the medical literature suggest that familial cases of CJD are consistent with an autosomal dominant mode of inheritance. In addition, in some extremely rare cases, CJD may take an infectious form. The disorder is thought to result from changes (mutations) in the gene that regulates the production of the human prion protein or direct contamination (transmission) with abnormal prion protein in infected brain tissue.



A variant form of CJD (V-CJD) has been reported in the United Kingdom that affects younger people (median age at onset: 28 years) than does classic CJD. In 1996, experts suggested the possibility that this variant might be associated with consumption of beef from cows with a related infectious brain disorder known as Bovine Spongiform Encephalopathy (BSE) or "Mad Cow Disease." BSE was first identified in the UK in 1986 and the number of reported cases grew rapidly, peaking in the winter of 1992-93 when almost 1,000 new cases were reported each week. Later, BSE also began to appear in some other European countries. Scientific research and debate continue concerning the potential link between BSE and V-CJD. In addition, coordinated national and international efforts are in place concerning the prevention, study, and surveillance of BSE and CJD. In early December 2000, European Union agriculture ministers agreed upon new measures to combat the spread of mad cow disease, including incinerating any cow over 30 months of age that had not tested negative for BSE. (BSE is thought to become detectable and infectious when cattle are approximately 30 months old.)

Resources

Alzheimer's Association

225 N. Michigan Avenue

17th Floor

Chicago, IL 60601

USA

Tel: (312)335-8700

Fax: (866)699-1246

Tel: (800)272-3900

TDD: (312)335-5886

Email: info@alz.org

Internet: http://www.alz.org



Alzheimer's Disease Education and Referral Center

P.O. Box 8250

Silver Spring, MD 20907-8250

Tel: (301)495-3311

Fax: (301)495-3334

Tel: (800)438-4380

Email: adear@nia.nih.gov

Internet: http://www.nia.nih.gov/alzheimers



Centers for Disease Control and Prevention

1600 Clifton Road NE

Atlanta, GA 30333

Tel: (404)639-3534

Tel: (800)232-4636

TDD: (888)232-6348

Email: cdcinfo@cdc.gov

Internet: http://www.cdc.gov/



National Hospice and Palliative Care Organization

1731 King Street, Suite 100

Alexandria, VA 22314

USA

Tel: (703)837-1500

Fax: (703)837-1233

Tel: (800)658-8898

Email: nhpco_info@nhpco.org

Internet: http://www.nhpco.org



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



World Health Organization (WHO)

Avenue Appia 20

Geneva 27, 1211

Switzerland

Tel: 41227912111

Fax: 41227913111

Internet: http://www.who.int/en/



Creutzfeldt-Jakob Disease Foundation, Inc.

PO Box 5312

Akron, OH 44334

USA

Tel: (330)665-5590

Fax: (330)668-2474

Tel: (800)659-1991

Email: help@cjdfoundation.org

Internet: http://www.cjdfoundation.org



CJD Voice

Internet: http://www.cjdvoice.org



CJD Aware!

2527 South Carrollton Avenue

New Orleans, LA 70118-3013

USA

Tel: (504)861-4627

Email: info@cjdaware.com

Internet: http://www.cjdaware.com



National Prion Disease Pathology Surveillance Center

Institute of Pathology

Case Western Reserve University

2085 Adelbert Road, Room 418

Cleveland, OH 44106-4907

USA

Tel: (216)368-0587

Fax: (216)368-4090

Email: cjdsurv@cwru.edu

Internet: http://www.cjdsurveillance.com



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



UCSF Memory and Aging Center

350 Parnassus Avenue

Suite 905

San Francisco, CA 94117

Tel: (415)476-6880

Fax: (415)476-4800

Email: mgeschwind@memory.ucsf.edu

Internet: http://www.memory.ucsf.edu



C-Mac Informational Services, Inc.

120 Clinton Lane

Cookeville, TN 38501-8946

Tel: (931)268-1201

Email: caregiver_cmi@hotmail.com

Internet: http://www.caregivernews.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  4/7/2009

Copyright  1984, 1985, 1987, 1988, 1990, 1991, 1992, 1994, 1996, 1997, 1998, 1999, 2000, 2001, 2003, 2007, 2009 National Organization for Rare Disorders, Inc.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.

USNWR 2013-2014Magnet Hospital RecognitionConsumer Choice2014 Best DoctorsJoint Commission Report

Disclaimer: The information on this website is for general informational purposes only and SHOULD NOT be relied upon as a substitute for sound professional medical advice, evaluation or care from your physician or other qualified health care provider.