(Subacute Spongiform Encephalopathy; CJD)
Creutzfeldt-Jakob disease (CJD) is a deadly brain illness that leads to dementia that gets worse with time. CJD is rare.
It is not the same as Variant CJD (vCJD), which is caused by eating contaminated beef.
It is thought that CJD is caused by proteins in the body called prions. The ones that may cause CJD fold up in a way that is not normal.
Things that may raise the risk depend on the type of CJD. There are 3 subtypes:
- Sporadic CJD—more common in people aged 50 years and older
- Iatrogenic CJD—happens after things like brain tissue grafts and cornea transplants
- Familial CJD—happens to people in the same family
Symptoms may be:
- Loss of focus
- Problems making choices
- Problems moving, such as muscle spasms
- Eyesight problems
- Problems speaking
- Changes in personality
- Problems sleeping
The doctor will ask about your symptoms and health history. A physical exam will be done.
Blood tests will be done to rule out other causes of dementia.
These tests may be done to make the diagnosis:
- Electroencephalogram (EEG) to test the electrical activity of the brain
- Lumbar puncture to test the fluid around the brain and spinal cord
- MRI scan to view images of the brain
The only way to confirm the disease is through an autopsy after death.
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There is no cure for CJD. It is treated by managing symptoms with:
- Pain medicine
- Antiseizure medicine to treat problems like muscle spasms
There are infection control guidelines to prevent iatrogenic CJD. There are no guidelines to prevent other types of CJD.
Creutzfeldt-Jakob Disease Foundation, Inc.
National Institute of Neurological Disorders and Stroke
Public Health Agency of Canada
Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at:https://www.dynamed.com/condition/creutzfeldt-jakob-disease. Updated May 01, 2015. Accessed October 14, 2019.
Creutzfeldt-Jakob disease, classic (CJD). Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/prions/cjd/index.html. Updated October 9, 2018. Accessed October 14, 2019.
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 August 13, 2019. Accessed October 14, 2019.
Sikorska B, Knight R, et al. Creutzfeldt-Jakob disease. Adv Exp Med Biol. 2012;724:76-90.
Last reviewed September 2019 by EBSCO Medical Review Board Rimas Lukas, MD Last Updated: 8/18/2020