Creutzfeldt-jakob Disease

What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease, also called CJD, is a disease of the nervous system. The nervous system includes the brain, spine, and nerves. With CJD, the cells and tissues of the nervous system are damaged. Affected persons are often found to have sponge-like changes of the brain. This results in loss of mental, emotional and physical functions. CJD is often confused with other types of dementia, such as Alzheimer's disease.

What causes CJD?

CJD is caused by a form of protein known as prion. Prions are normally found in the cells and tissues of many body organs including the brain. The abnormal form of prion affects the other proteins, causing them to change their shape. The change in the shape makes the abnormal prion clump together inside the nervous system. This clumping causes nerve cells to die.

What are the different types of CJD?

There are four different types of CJD:

  • Sporadic CJD: This is the most common type of CJD. Caregivers do not know exactly what causes this type of CJD. It is most common in people 50 to 70 years of age. It affects the ability to think, learn and remember. Movement and balance is also affected.

  • Variant CJD: This new type of CJD was first seen in the United Kingdom. This was probably caused by eating meat from cows with bovine spongiform encephalopathy or BSE. This is also related to mad cow disease. Problems in behavior, mood and the ability to relate to others are common.

  • Familial CJD: This occurs when a parent with CJD passes the disease to his children.

  • Iatrogenic CJD: You can get this type of CJD from organ transplants or contaminated (dirty) surgical instruments. It may also be passed from drugs made from affected human tissues.

What are the signs and symptoms of CJD?

CJD progresses quickly from the time signs and symptoms begin to the time they worsen. The signs and symptoms depend on the type of CJD you may have. This may include any of the following:

  • Delirium, dementia (memory problems), or hallucinations (seeing or hearing things that are not real).

  • Muscle twitching or sudden, painful muscle movements.

  • Nervousness or anxiety.

  • Personality or behavior changes, such as confusion, depression and mood changes.

  • Problems in posture, balance, or walking.

  • Sleepiness.

  • Speech or swallowing problems.

How is CJD diagnosed?

You may need one or more of the following tests:

  • Biopsy: This is a procedure where a small piece of tissue from your brain or tonsils is collected and sent to the lab for tests.

  • EEG: This test is also called an electroencephalogram. Many small pads or metal discs are put on your head. Each has a wire that is hooked to a machine. This machine prints a paper tracing of brain wave activity from different parts of your brain. Caregivers look at the tracing to see how your brain is working.

  • Lumbar puncture: This procedure may also be called a spinal tap. During a lumbar puncture, you will need to lie very still. Caregivers may give you medicine to make you lose feeling in a small area of your back. Caregivers will clean this area of your back. A needle will be put in, and fluid removed from around your spinal cord. The fluid will be sent to a lab for tests. The tests check for infection, bleeding around your brain and spinal cord, or other problems. Sometimes medicine may be put into your back to treat your illness.

  • MRI: This scan uses powerful magnets and a computer to take pictures of your brain. It will also take pictures of the blood vessels and structures in your head. You may be given dye, also called contrast, before the test. Tell caregivers if you are allergic to dye, iodine, or seafood. Remove all jewelry, and tell caregivers if you have any metal in or on your body. Metal can cause serious injury. Tell caregivers if you cannot lie still or are anxious or afraid of closed spaces.

How is CJD treated?

There is no specific treatment for CJD, but medicine and other treatments are used to decrease signs and symptoms of the disease. Treatments may include medicines that control pain, anxiety, and muscle spasms. Speech or swallowing therapies, and preparation for long term care may also be needed.

Where can I find support and more information?

CJD is a life-changing condition for you and your family. Accepting that you have CJD is hard. You and those close to you may feel angry, sad, or confused. These feelings are normal. Talk to your caregivers, family, or friends about your feelings. You may also want to join a support group with other people who have CJD. Ask your caregiver for contact information for support groups. Contact the following for more information:

  • National Institute of Neurological Disorders and Stroke
    P.O. Box 5801
    Bethesda , MD 20824
    Phone: 1- 301 - 496-5751
    Phone: 1- 800 - 352-9424
    Web Address: http://www.ninds.nih.gov
  • Centers for Disease Control and Preventions
    1600 Clifton Rd.
    Atlanta , GA 30333
    Phone: 1- 800 - 232-4636
    Web Address: http://www.cdc.gov

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2013 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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