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Creutzfeldt-Jakob Disease
(Subacute Spongiform Encephalopathy)

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

There are four types of CJD:

  • Sporadic (classical) CJD – most common type; usually affects people age 50 and older.
  • Familial CJD – an inherited form of the disease.
  • Iatrogenic CJD – contracted through medical procedures, such as injections of growth hormone.
  • New Variant CJD (nvCJD) – a relatively new form of CJD. It is caused by eating contaminated beef products, which may cause bovine spongiform encephalopathy (BSE). (BSE is commonly known as mad cow disease.) nvCJD differs from other forms of CJD, because it affects younger people and has a longer average time course.
Causes:
There has been a great deal of scientific research and debate about the cause of CJD. Today, it is generally believed that CJD is caused by infectious proteins called prions. Prions can transform normal protein molecules into abnormal, disease-causing molecules.

Risk Factors:
A risk factor is something that increases your chances of getting a disease or condition.
  • Age: CJD most commonly occurs in people ages 50-75
  • Use of cadaveric growth hormone
  • Cornea transplants
  • Dura mater grafts
  • Family members with CJD
  • Eating beef products produced by countries with an epidemic of BSE (mad cow disease)
Symptoms:
Initially, there are no symptoms. As CJD progresses, symptoms that may occur include:
  • Memory lapses
  • Difficulty concentrating
  • Impaired judgement
  • Loss of coordination
  • Blurred vision
  • Behavior and mood changes
  • Muscle spasms
  • Seizures
  • Loss of mental and physical function

Depending on the type of CJD, the disease may last from 3 –12 months, or occasionally longer, and is almost always fatal.

Diagnosis:
Your doctor will ask about your symptoms and medical history, and perform a physical exam.

CJD is a difficult disease to diagnose, as there is no single test for detection. The following tests may be used to help make a Diagnosis:

  • Electroencephelogram (EEG) – a test that records the brain's activity by measuring electrical currents through the brain.
  • CT Scan – a type of x-ray that uses a computer to make pictures of the inside of the body, in this case the brain
  • MRI Scan – a test that uses magnetic waves to make pictures of the inside of the body, in this case the brain
  • Brain Biopsy – removal of a sample of brain tissue for testing
Treatment:
There is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms.

Drug therapy may include:

  • Opiate medication to treat pain
  • Anticonvulsive drugs to help decrease jerking (myoclonus) or other neuromuscular problems
Prevention:
To avoid nvCJD, it is recommended that you avoid eating beef produced in areas that may have BSE (mad cow disease). There are no known ways to prevent other forms of CJD.
 
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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