Pronounced: Kroytz-felt Jay-kob
En Espaol (Spanish Version)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) CJDmost common type; usually affects people age 50 and older
- Familial CJDan inherited form of the disease
- Iatrogenic CJDcontracted 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) 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 most noninherited cases are caused by infectious proteins called prions. Prions can transform normal protein molecules into abnormal, disease-causing molecules.
Risk FactorsA risk factor is something that increases your chance of getting a disease or condition. Risk factors include: Age: CJD most commonly occurs in people ages 50 to 75.Use of cadaveric growth hormoneCornea transplantsDura mater graftsFamily 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 Symptoms Initially, there are no symptoms. As CJD progresses, symptoms that may occur include: Memory lapsesDifficulty concentratingImpaired judgementDifficulty with speechLoss of coordinationBlurred visionBehavior and mood changesMuscle spasmsSeizuresLoss of mental and physical functionDepending on the type of CJD, the disease may last from 3 to 36 months, or occasionally longer, and is almost always fatal. DiagnosisYour 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: 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 Alzheimers disease . MRI Scan of the Brain 2008 Nucleus Medical Art, Inc.
TreatmentThere is no cure for CJD. The aim of treatment is to relieve pain and alleviate symptoms. Drug therapy may include: Opiate medication to treat painAnticonvulsive drugs to help decrease neuromuscular problems PreventionTo avoid the new variant form of this condition, 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. RESOURCES: Creutzfeldt-Jakob Disease Foundation, Inc.http://www.cjdfoundation.org National Institute of Neurological Disorders and Strokehttp://www.ninds.nih.gov World Health Organizationhttp://www.who.org CANADIAN RESOURCES: Canadian Health Networkhttp://www.canadian-health-network.ca/ Public Health Agency of Canadahttp://www.phac-aspc.gc.ca References: Churg-Strauss syndrome. EBSCO Publishing Dynamed website. Available at: http://www.ebscohost.com/dynamed . 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: The McGraw-Hill Companies; 2005.
Roos RP. Creutzfeldt-Jakob disease. In: Gilman S, ed. MedLink Neurology. San Diego, CA: MedLink Corporation. Available at: http://www.medlink.com . Accessed February 23, 2008. Last reviewed February 2008 by J. Thomas Megerian, MD, PhD, FAAPPlease 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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