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, or 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
Your doctor will ask 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. Tests may include: Blood testsElectroencephalogram
(EEG) to record the electical activity of the brain
Cerebrospinal fluid analysis
Tonsillar biopsy and brain
Imaging tests take pictures of your internal body structures. Imaging tests may include: MRICT 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.
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CDC's diagnostic criteria for Creutzfeldt-Jakob Disease (CJD), 2010. Centers for Disease Control and Prevention website. Available at:
. Updated August 26, 2010. Accessed June 27, 2013.
Mastrianni JA. The genetics of prion disease.
Patry D, Curry B, Easton D, Mastrianni JA, Hogan DB. Creutzfeld-Jakob disease (CJD) after blood product transfusion from a donor with CJD.
Rinne ML, McGinnis SM, Samuels MA, Katz JT, Loscalzo J. Clinical problem-solving. A startling decline.
N Engl J Med
Last reviewed June 2013 by Rimas Lukas, MD; Brian Randall, 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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