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Variant Creutzfeldt-Jakob Disease

Authors:
  • Cynthia M. Johnson, MA
Publication Type:

Condition

Variant Creutzfeldt-Jakob Disease

(Human Mad Cow Disease; vCJD)

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. BSE can spread to humans and 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 buildup of these prions may be linked to vCJD.

The Nervous System.

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Risk Factors

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

  • Eating beef from infected cows
  • Getting a blood transfusion from a person who had the disease

Symptoms

After a person is exposed, it can take up to 20 years until they feel sick. Problems get worse over time:

  • Early phase (0 to 6 months)—mental health problems, such as depression, anxiety, withdrawal, memory problems, and trouble speaking
  • Middle phase—nervous system problems, such as difficulty with walking and coordination, muscle jerking and stiffness, and a hard time speaking
  • Late phase—being unable to talk or move

Diagnosis

The doctor will ask about symptoms and health history. A physical exam will be done.

These tests may be done to learn more about the brain:

  • Blood tests
  • Electroencephalogram (EEG) to record the electrical activity of the brain
  • to test the fluid that protects the brain and spinal cord

Images may be taken with:

Brain tissue may also be looked at with:
  • A brain biopsy
  • An autopsy done after a person has died

Treatment

The goals of treatment are to offer support and ease discomfort. There is no cure for vCJD.

Prevention

The risk of vCJD may be lowered by not eating beef products when traveling to places where BSE is a problem.

RESOURCES:

CANADIAN RESOURCES:

CANADIAN RESOURCES:

References

  • Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at: https://www.dynamed.com/condition/creutzfeldt-jakob-disease.
  • Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: https://www.ninds.nih.gov/creutzfeldt-jakob-disease-fact-sheet.
  • Geschwind, M.D. Prion Diseases. Continuum (Minneap Minn). 2015; 21 (6 Neuroinfectious Disease): 1612-1638.
  • Mackenzie, G. and Will, R. Creutzfeldt-Jakob disease: recent developments. F1000 Research, 2017; 6: 2053.
  • Shir, D., Lazar, E.B., et al. Analysis of clinical features, diagnostic tests, and biomarkers in patients with suspected Creutzfeldt-Jakob Disease, 2014-2021. JAMA Netw Open, 2022; 5(8): e2225098.

Contributors

  • Rimas Lukas, MD
Last Updated:
2022-09-01

(C) Copyright 2022 EBSCO Information Services

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.