Source: Nightbreed. Creutzfeldt-Jakob disease (CJD) is a degenerative, fatal brain disorder. CJD usually runs a rapid course. Typically 70 percent of individuals die within one year. In the early stages of the disease, people 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.
CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. A prion—derived from “protein” and “infectious”—causes CJD in people and TSEs in animals. Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges when examined under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).
CJD and sporadic fatal insomnia (sFI), overall they are characterized by rapidly progressive dementia. Initially, individuals experience problems with muscle coordination, personality changes (including impaired memory, judgment, and thinking), and impaired vision. People with the disease, especially with FFI, also may experience insomnia, depression, or unusual sensations. As the illness progresses, peoples’ mental impairment becomes severe. They often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak, and enter a coma.
Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s and Huntington’s disease. However, CJD causes unique changes in brain tissue which can be seen at autopsy. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia.
Current scientific consensus maintains that abnormal forms of normal cellular proteins called prions cause CJD in people. CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Spouses and other household members of people with sporadic CJD have no higher risk of contracting the disease than the general population. However, exposure to brain tissue and spinal cord fluid from infected persons should be avoided to prevent transmission of the disease through these materials.
Many people are concerned that it may be possible to transmit CJD through blood and related blood products such as plasma. Some animal studies suggest that contaminated blood and related products may transmit the disease.
Studies have found that infectious prions from BSE and vCJD accumulate in the lymph nodes (which produce white blood cells), the spleen, and the tonsils. At present, four cases of vCJD infection have been identified following transfusion of red blood cells from asymptomatic donors who subsequently died from vCJD. Recently, one case of likely transmission of vCJD infection by concentrates of blood-clotting protein has been reported in an elderly individual with hemophilia in the United Kingdom.
Currently, there is no treatment that can cure or control CJD, although studies of a variety of drugs are now in progress. Current treatment for CJD is aimed at easing symptoms and making the person as comfortable as possible. Opiate drugs can help relieve pain if it occurs, and the drugs clonazepam and sodium valproate may help relieve myoclonus. During later stages of the disease, intravenous fluids and artificial feeding also may be used.
