Creutzfeldt-Jakob disease (CJD) is a brain disorder caused by prions
that induce abnormal folding of normal brain proteins. About 85 percent of CJD cases occur sporadically and have unknown origins. The remaining cases are either familial (caused by inherited gene mutations) or iatrogenic (caused by contaminated instruments used during medical treatments or by transplants from infected organ donors). Most CJD cases are seen in people over the age of 50. Like most prion diseases, degeneration occurs rapidly after the onset of the disease and death usually occurs within 1 year. A variant of CJD (vCJD) affects people at a younger age, with the median age of death being 28 years. The most likely cause of vCJD is exposure, probably through contaminated food, to bovine spongiform encephalopathy—a disease of cattle commonly called “mad cow disease.”
CJD patients undergo rapid personality changes and dementia. Neurological signs, such as unsteadiness and involuntary movement, appear early on. Symptoms vary slightly in vCJD, which is characterized by depression and occasional psychosis. Neurological symptoms do not develop until later in the disease progression. Both variations of the disease are accompanied by symptoms such as blurred vision, muscle stiffness, sleepiness, speech impairment, confusion, and lack of coordination.
Currently there is no cure for CJD. Certain medications, such as interleukins, can slow the disease’s progression. As with other cases of dementia, counseling and behavior modification may help patients control dangerous or inappropriate behaviors.
There is no known way to prevent sporadic or familial CJD. Hospitals have specific procedures and regulations regarding transfusions and equipment usage in order to prevent iatrogenic CJD. Strict regulations on food supply, including regulation of animal feed and good surveillance systems for tracking cattle health, can prevent the spread of vCJD.