Variant Creutzfeldt-Jakob Disease

(Human Mad Cow Disease; vCJD)


Variant Creutzfeldt-Jakob disease (vCJD) is a type of prion disease that can cause death. Bovine spongiform encephalopathy (BSE) is a prion disease in cows. There is evidence that BSE can spread to humans. This results in vCJD. It is also known as mad cow disease.


It is believed that vCJD is caused by proteins called prions. Prions are normal proteins in the body. These prions may fold up in a way that is not normal. This can change them into the protein that causes the illness. The build up of these prions may be linked to from vCJD.

The Nervous System

Nucleus factsheet image
Copyright © Nucleus Medical Media, Inc.

Risk Factors

Variant CJD is more common in younger people. Being around tissue that contains prions may raise your risk. This may happen from:

  • Eating beef from infected cows
  • Receiving a blood transfusion from someone who had the disease


After you are exposed, it can take up to 20 years until you feel sick. Symptoms can follow these three phases:

  • Early phase (0 to 6 months)—mental health problems, such as depression, anxiety, withdrawal, memory problems, and difficulty pronouncing words
  • Middle phase—neurological problems, such as problems with walking and coordination, muscle jerks and stiffness, and a hard time speaking
  • Late phase—inability to talk or move


Your doctor will ask about your symptoms and health history. A physical exam will be done. You may have:

  • Blood tests
  • Electroencephalogram (EEG) to record the electrical activity of the brain
  • Lumbar puncture —to test the fluid that protects the brain and spinal cord
  • Brain biopsy
  • Tonsillar biopsy

Pictures may be taken with:

In many cases, a finding is not reached until an autopsy is done after death.


There is no cure for vCJD. You will receive support and help easing discomfort.


To lower your chance of getting vCJD, do not eat beef products. Avoid processed meat like sausages and hot dogs and beef items that contain brain, spinal cord, or bone marrow.


Creutzfeldt-Jakob Disease Foundation, Inc.
National Institute of Neurological Disorders and Stroke


Public Health Agency of Canada


Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010;23(4):277-298.
Creutzfeldt-Jakob disease. EBSCO DynaMed Plus website. Available at: . Updated May 1, 2015. Accessed June 25, 2018.
Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at:
...(Click grey area to select URL)
Updated May 9, 2018. Accessed June 25, 2018.
Dawidowska K. Where’s the (safe) beef? Prevention. 2004;56:34.
Hill AF, Butterworth RJ, et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet. 1999;353(9148):1183-1189.
Holman RC, Belay ED, Christensen KY, et al. Human prion diseases in the United States. PLoS One. 2010;5(1):e8521.
Mackenzie G, Will R. Creutzfeldt-Jakob disease: recent developments. F1000res. 2017;6:2053.
Moo-ve over, beef burgers: EN finds many alternatives. Environmental Nutrition. 2004;27:5.
Patry D, Curry B, et al. Creutzfeldt-Jakob disease (CJD) after blood product transfusion from a donor with CJD. Neurology. 1998;50(6):1872-1873.
Prusiner SB. Detecting mad cow disease. Sci Am. 2004;291(1):60-67.
Raloff J. Better protection from mad cow disease. Science News. 2004;165:93.
Rinne ML, McGinnis SM, Samuels MA, Katz JT, Loscalzo J. Clinical problem-solving. A startling decline. N Engl J Med. 2012;366(9):836-842
Smith-Bathgate B. Creutzfeldt-Jakob disease: diagnosis and nursing care issues. Nursing Times. 2005;101(20):52-53.
Variant Creutzfeldt-Jakob disease (vCJD). Centers for Disease Control and Prevention website. Available at:
...(Click grey area to select URL)
Updated February 6, 2015. Accessed June 25, 2018.
Zeidler M, Sellar R, Collie DA, et al. The pulvinar sign on magnetic resonance imaging in variant Creutzfeldt-Jakob disease. Lancet. 2000;355(9213):1412-1419.
Last reviewed May 2018 by EBSCO Medical Review Board Rimas Lukas, MD
Last Updated: 6/25/2018

EBSCO Information Services is fully accredited by URAC. URAC is an independent, nonprofit health care accrediting organization dedicated to promoting health care quality through accreditation, certification and commendation.

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

To send comments or feedback to our Editorial Team regarding the content please email us at Our Health Library Support team will respond to your email request within 2 business days.