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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.

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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.

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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
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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
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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

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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
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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.