(Subacute Spongiform Encephalopathy; CJD)
Pronounced: Kroytz-felt Jay-kob
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 or receipt of ]]>corneal transplants]]> or dura mater implants from affected donors
- ]]>New variant CJD (nvCJD)]]> —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.
There has been a great deal of scientific research and debate about the cause of CJD. Today, it is generally believed that most noninherited cases are caused by infectious proteins called prions. Prions can transform normal protein molecules into abnormal, disease-causing molecules.
These factors increase your chance of developing CJD. Tell your doctor if you have any of these risk factors:
- Age: CJD most commonly occurs in people aged 50-75
- Use of cadaveric growth hormone
- Cornea transplants
- Dura mater grafts
- Family members with CJD (Approximately 5%-10% of cases are inherited.)
- Eating beef products produced by countries with an epidemic of BSE (mad cow disease)
- Healthcare workers who work with brain tissues
Initially, there are no symptoms. As CJD progresses, symptoms that may occur include:
- Memory lapses
- Difficulty concentrating
- Impaired judgement
- Difficulty with speech
- Loss of coordination
- Blurred vision
- Behavior and mood changes
- Muscle spasms
- Loss of mental and physical function
Depending on the type of CJD, the disease may last from 3-36 months, or occasionally longer, and is almost always fatal.
Your doctor will ask about your symptoms and medical history, and perform a physical exam.
CJD is a difficult disease to diagnose. There is no single test for detection. The following tests may be used to help make a diagnosis:
- Electroencephalogram (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
- Blood tests and ]]>lumbar puncture]]> —to obtain cerebrospinal fluid; This may be helpful in distinguishing CJD from other diseases with similar symptoms, such as ]]>Alzheimer’s disease]]> .
MRI Scan of the Brain
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 neuromuscular problems
Creutzfeldt-Jakob Disease Foundation, Inc.
National Institute of Neurological Disorders and Stroke
World Health Organization
Public Health Agency of Canada
Churg-Strauss syndrome. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated December 12, 2007. Accessed February 27, 2008.
Creutzfeldt-Jakob Disease Foundation, Inc. website. Available at: http://www.cjdfoundation.org .
Kasper DL, Braunwald E, Fauci AS, et al. Harrison's Principles of Internal Medicine . 16th ed. New York, NY: McGraw-Hill; 2005.
Roos RP. Creutzfeldt-Jakob disease. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. Medline website. Available at: http://www.medlink.com . Accessed February 23, 2008.
Last reviewed January 2009 by ]]>J. Thomas Megerian, MD, PhD, FAAP]]>
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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