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Creutzfeldt-Jakob Disease

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

Condition

Creutzfeldt-Jakob Disease

(Subacute Spongiform Encephalopathy; CJD)

Definition

Creutzfeldt-Jakob disease (CJD) is a deadly brain illness. It leads to dementia that gets worse with time. CJD is rare.

It is similar to, but not exactly the same as Variant CJD (vCJD), which is caused by eating contaminated beef.

Causes

CJD is caused by proteins in the body called prions. The prions that may cause CJD fold up in a way that is not normal. They cause other proteins near them to start folding in the same way, and this leads to problems in the brain.

Risk Factors

Things that may raise the risk depend on the type of CJD. There are various subtypes:

  • Sporadic CJD—more common in people at least 50 years of age
  • Iatrogenic CJD—happens after procedures such as brain tissue grafts and cornea transplants
  • Familial CJD—happens to people in the same family

Symptoms

A person with CJD may have:

  • Forgetfulness
  • Loss of focus
  • Problems making choices
  • Problems moving or muscle spasms called myoclonic jerks
  • Eyesight problems
  • Problems speaking
  • Changes in personality
  • Problems sleeping

Diagnosis

The doctor will ask about 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 help make the diagnosis:

  • Electroencephalogram (EEG) to test the brain's electrical activity
  • Lumbar puncture to test the fluid around the brain and spinal cord
  • MRI scan to view images of the brain
  • PET scan to look at how the brain is working
  • Biopsy to look for certain problems in the brain
The only way to confirm the disease is with an autopsy after death.
MRI Scan of the Brain.

Copyright © 2002 Nucleus Communications, Inc. All rights reserved.

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Treatment

The goal of treatment is to manage symptoms. There is no cure for CJD. The doctor may give:

  • Pain medicine
  • Antiseizure medicine to treat problems like muscle spasms

Prevention

There are infection control guidelines to prevent iatrogenic CJD. There are no guidelines to prevent other types of CJD.

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, classic (CJD). Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/prions/cjd/index.html.
  • Creutzfeldt-Jakob disease fact sheet. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/health-information/patient-caregiver-education/fact-sheets/creutzfeldt-jakob-disease-fact-sheet.
  • Uttley, L., Carroll, C., et al. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. The Lancet Infectious Diseases, 2020; 20 (1): E2-E10.

Contributors

  • Rimas Lukas, MD
Last Updated:
2023-06-01

(C) Copyright 2023 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.