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Creutzfeldt-Jakob Disease

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BC: Citations: | http://www.medicinenet.com/creutzfeldt-jakob_disease/page2.htm http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001792/ http://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_disease

FC: Creutzfeldt-Jakob Disease

1: INDEX 1.Description of Disorder 2.Anatomy of Disorder 3.Symptoms 4.Risk Factors and Prevention of the Disorder 5.Test and Diagnosis 6.Medical Treatment and Prognosis 7.Glossary (Citations)

2: Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. It affects about one person in every one million people per year worldwide. in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, symptoms occurs about age 60, and about 90 percent of patients die within 1 year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. | DESCRIPTION OF DISORDER

4: Creutzfeldt-Jakob Disease (CJD) is a form of progressive dementia characterized by loss of nerve cells and degeneration of nerve cell membranes leading to the production of small holes in the brain. It is rare, degenerative, and fatal. Related to "mad cow disease," CJD usually has rapid symtpoms and decline. | ANATOMY OF DISORDER

5: There are three major categories of CJD: In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85 percent of cases. In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10 percent of cases of CJD in the United States are hereditary. In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. fewer than 1 percent of cases have been acquired CJD.

6: SYMPTOMS | Early symptoms include lapses in memory, mood swings similar to depression, lack of interest and social withdrawal. The person may become unsteady on his/her feet. Later symptoms may include blurred vision, sudden jerking movements and rigidity in the limbs. The person may experience slurred speech and have difficulty swallowing. Eventually, movement and speech are lost.

8: RISK FACTORS & PREVENTION OF THE DISORDER | Some researchers believe another "slow virus" or another organism causes CJD. However, they have never been able to isolate a virus or other organism in people with the disease. Furthermore, whatever it is that causes CJD has several characteristics that are unusual for organisms such as viruses and bacteria. It is difficult to kill, it does not appear to contain any genetic information in the form of DNA and it usually has a long incubation period before symptoms appear. (Meaning it can stay in the body for 40 years dormant before it shows symtpms) | ---> Prion proteins occur in both a normal form, which is a harmless protein and in an infectious form. The harmless and infectious forms of the prion protein have the same amount of amino acids but the infectious form ofthe protein takes a different shape than the normal protein. Sporadic CJD may develop because some of a person's normal prions spontaneously change into the infectious form of the protein | CJD cannot be transmitted through the air or through touching or most other forms of casual contact. | The leading scientific theory at this time maintains that CJD is caused by a type of protein called a prion.

9: To reduce the already very low risk of CJD transmission from one person to another, people should never donate blood, tissues, or organs if they have suspected or confirmed CJD, or if they are at increased risk because of a family history of the disease.

10: TEST & DIAGNOSIS | There is currently no single diagnostic test for CJD. When a doctor suspects CJD, the first concern is to rule out treatable forms of dementia | The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neuropatholoist

12: MEDICAL TREATMENT AND PRONOSIS

13: There is no treatment that can cure or control CJD. Researchers have tested many drugs. Current treatment for CJD is aimed at alleviating symptoms and making the patient as comfortable as possible. During later stages of the disease, changing the person's position frequently can keep him or her comfortable and helps prevent bedsores. A catheter can be used to drain urine if the patient cannot control bladder function, and fluids and artificial feeding also may be used

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