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Creutzfeldt-Jakob Disease(Subacute Spongiform Encephalopathy; CJD)

Pronounced: Kroytz-felt Jay-kob

Definition

Creutzfeldt-Jakob disease (CJD) is a rare, fatal, degenerative brain disorder characterized by rapidly progressive dementia.

CJD can be categorized into different subtypes:

  • Sporadic CJD—most common type; usually affects people aged 50 years and older
  • Familial CJD—an inherited form of the disease
  • Iatrogenic CJD—contracted through medical procedures, such as injections of growth hormone, or receipt of corneal transplants or dura mater implants from affected donors

Variant CJD (vCJD) is a different prion disease caused by eating contaminated beef products. This variant form differs from other forms of CJD because it affects younger people and has a longer average time course.

Causes

It is generally believed that CJD is caused by infectious proteins called prions. Prions are normal proteins in the body. If these prions fold up in a different way than normal, they may transform into the protein that causes the illness. The build-up of abnormal prions may be linked to the brain damage associated with CJD.

Risk Factors

People over 50 years old have an increased chance of getting sporadic CJD.

Family members with CJD increase your chance of getting familial CJD. Approximately 10% of cases are inherited.

Factors that may increase your chance of getting iatrogenic CJD include:

  • Use of cadaveric growth hormone
  • Cornea transplants
  • Dura mater grafts
  • Healthcare workers who work with brain tissues
  • Blood transfusion from someone with CJD
Symptoms

Symptoms may include:

  • Memory lapses
  • Difficulty concentrating
  • Impaired judgment
  • Difficulty with speech
  • Loss of coordination
  • Blurred vision
  • Behavior and mood changes
  • Muscle spasms
  • Seizures
  • Loss of mental and physical function
Diagnosis

Your doctor will ask about your symptoms and medical history. A physical exam will be done.

CJD is a difficult disease to diagnose. There is no single test to detect it. Tests may include:

  • Blood tests
  • Electroencephalogram (EEG) to record the electrical activity of the brain
  • Cerebrospinal fluid analysis
  • Brain biopsy
  • Tonsillar biopsy and brain biopsy

Imaging tests take pictures of your internal body structures. Imaging tests may include:

In many cases, final diagnosis requires an autopsy after death.

MRI Scan of the Brain

MRI of the Brain

Copyright © Nucleus Medical Media, Inc.

Treatment

There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.

Your doctor may recommend:

  • Prescription pain relievers
  • Anticonvulsive medication for neuromuscular symptoms
Prevention

There are no current guidelines to prevent sporadic CJD.

If you have a family history of CJD, consider talking to a genetic counselor to better understand your risk.

The World Health Organization and Centers for Disease Control and Prevention have strict infection control guidelines to prevent iatrogenic CJD.

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
http://www.hc-sc.gc.ca

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

References:

About CJD (Creutzfeldt-Jakob disease, classic). Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncidod/dvrd/cjd/index.htm. Updated December 11, 2013. Accessed May 30, 2014.

Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol. 2010;23(4):277-298.

CDC's diagnostic criteria for Creutzfeldt-Jakob Disease (CJD), 2010. Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncidod/dvrd/cjd/diagnostic_criteria.html. Updated August 26, 2010. Accessed May 30, 2014.

Creutzfeldt-Jakob disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com. Updated October 28, 2013. Accessed May 30, 2014.

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 May 22, 2014. Accessed May 30, 2014.

Mastrianni JA. The genetics of prion disease. Genet Med. 2010;12(4):187-195.

Patry D, Curry B, et al. Creutzfeld-Jakob disease (CJD) after blood product transfusion from a donor with CJD. Neurology. 1998;50(6):1872-1873.

Rinne ML, McGinnis SM, et al. Clinical problem-solving. A startling decline. N Engl J Med. 2012;366(9):836-842.



Last reviewed May 2014 by Rimas Lukas, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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