Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive
CJD can be categorized into different subtypes: Sporadic CJD—most common type; usually affects people aged 50 years and olderFamilial CJD—an inherited form of the disease
Iatrogenic CJD—contracted through medical procedures, such as injections of growth hormone, receipt of
or dura mater implants from affected donors
is a different prion disease caused by eating contaminated beef products. This variant form differs from other forms of CJD because it affects younger people and has a longer average time course.
It is generally believed that CJD is caused by infectious proteins called prions. Prions are normal proteins in the body. If these prions fold up in a different way than normal, they may transform into the protein that causes the illness. The build-up of abnormal prions may be linked to the brain damage associated with CJD.
People over 50 years old have an increased chance of getting sporadic CJD.
Family members with CJD increase your chance of getting familial CJD. Approximately 10% of cases are inherited.
Factors that may increase your chance of getting iatrogenic CJD include: Use of cadaveric growth hormoneCornea transplantsDura mater graftsHealthcare workers who work with brain tissuesBlood transfusion from someone with CJD
Symptoms may include: Memory lapsesDifficulty concentratingImpaired judgmentDifficulty with speechLoss of coordinationBlurred visionBehavior and mood changesMuscle spasmsSeizuresLoss of mental and physical function
You will be asked about your symptoms and medical history. A physical exam will be done.
CJD is a difficult disease to diagnose. There is no single test to detect it.
Your bodily fluids may be tested. This can be done with: Blood testsCerebrospinal fluid analysis
Tonsillar biopsy and brain
The electrical activity of your brain may be tested. This can be done with an electroencephalogram
Images may be taken of your bodily structures. This can be done with: MRI scanCT scanSPECT imaging
In many cases, final diagnosis requires
MRI Scan of the Brain
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There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.
Your doctor may recommend: Prescription pain relieversAnticonvulsive medication for neuromuscular symptoms
There are no current guidelines to prevent sporadic CJD.
If you have a family history of CJD, consider talking to a genetic counselor to better understand your risk.
The World Health Organization and Centers for Disease Control and Prevention have strict infection control guidelines to prevent iatrogenic CJD.
About CJD (Creutzfeldt-Jakob disease, classic). Centers for Disease Control and Prevention website. Available at:
http://www.cdc.gov/ncidod/dvrd/cjd/index.htm. Updated December 11, 2013. Accessed September 17, 2014.
Brown K, Mastrianni JA. The prion diseases.
J Geriatr Psychiatry Neurol. 2010;23(4):277-298.
CDC's diagnostic criteria for Creutzfeldt-Jakob Disease (CJD), 2010. Centers for Disease Control and Prevention website. Available at:
http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html. Updated August 26, 2010. Accessed September 17, 2014.
Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at:
http://www.ebscohost.com. Updated August 28, 2014. Accessed September 17, 2014.
Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at:
http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm. Updated May 22, 2014. Accessed September 17, 2014.
Mastrianni JA. The genetics of prion disease.
Genet Med. 2010;12(4):187-195.
Patry D, Curry B, et al. Creutzfeld-Jakob disease (CJD) after blood product transfusion from a donor with CJD.
Rinne ML, McGinnis SM, et al. Clinical problem-solving. A startling decline.
N Engl J Med.
Last reviewed August 2014 by Rimas Lukas, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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