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Variant Creutzfeldt-Jakob Disease

(Human Mad Cow Disease; vCJD)

by Cynthia M. Johnson, MA

Home | Health Information Center | Disease, Condition, & Injury Fact Sheets | Article

Definition

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.

Causes

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

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

Symptoms

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

Diagnosis

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:

MRI scan
CT scan
SPECT scan

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

Treatment

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

Prevention

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.

RESOURCES:

Creutzfeldt-Jakob Disease Foundation, Inc.
http://www.cjdfoundation.org

National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov

CANADIAN RESOURCES:

Health Canada
https://www.canada.ca

Public Health Agency of Canada
http://www.phac-aspc.gc.ca

References:

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Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at:
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Updated May 9, 2018. Accessed June 25, 2018.

Dawidowska K. Where’s the (safe) beef? Prevention. 2004;56:34.

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Moo-ve over, beef burgers: EN finds many alternatives. Environmental Nutrition. 2004;27:5.

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Variant Creutzfeldt-Jakob disease (vCJD). Centers for Disease Control and Prevention website. Available at:
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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

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