Complete Information on Creutzfeldt Jakob disease

Creutzfeldt-Jakob disease (CJD) is aform of brain damage that causes a rapid decrease of mental function and movement. It isbelieved to result froma protein called a prion. It affects about one person in every one million people per year worldwide. It usually first appears between ages 20 and 70, with average age at onset of symptoms in the late 50s. CJD can be grouped into classic or new variant disease. About 15% of cases of CJD have a family history that demonstrates an inherited form of the illness (autosomal dominant transmission). Onset of disease is significantly earlier in familial cases.

There are three major forms of CJD. In sporadic CJD, the disease shows equable though the person has no known risk factors for the disease. In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation relatedwith CJD. In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. CJD is characterized by rapidly progressive dementia. People with the disease also may experience insomnia, depression, or unusual sensations.

CJD does not cause a fever or other flu-like symptoms. The symptoms of CJD are caused by the progressive death of the brain's nerve cells, which is associated with the build-up of abnormal prion proteins. When brain tissue from a CJD patient is examined under a microscope, many tiny holes can be seen where whole areas of nerve cells have died. There is no treatment that can cure or control CJD. Current treatment for CJD is opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus. A catheter can be used to drain urine if the patient cannot control bladder function.

Intravenous fluids and artificial feeding also may be used. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors. These include sedatives, antipsychotics, and others. Supportive treatments can make life more comfortable for those affected. Family counseling may help in coping with the changes required for home care. Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. Persons who have a medical history of diagnosed or possible CJD are not used as cornea donors.