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Creutzfeldt Jakob Disease

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@alzheimers.org
Internet: http://www.alzheimers.org

Center 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
Email: me20t@nih.gov
Internet: http://www.ninds.nih.gov/

World Health Organization (WHO)
Avenue Appia 20
Geneva 27, 1211
Switzerland
Tel: + 41 22 791 21 11
Fax: + 41 22 791 31 11
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/

Human BSE Foundation
Matfen Court
Chester Le Street
County Durham, DH2 2TX
UK
Tel: 0191 389 4157
Email: info@hbsef.org
Internet: http://www.hbsef.org

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.

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