Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive viral disease that attacks the central nervous system, causing dementia and neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbance, and paralysis. Adults from the ages of 40 to 65 are usually affected by this condition. Males and females are affected equally, and this condition is always fatal.
In 1996, a new variant of CJD emerged in Europe. The new variant (nvCJD), also known as “mad cow disesase,” is a rare, fatal neurodegenerative disease. Most cases of nvCJD have been reported in the United Kingdom, but cases have also
been reported in Canada, Ireland, Italy, and the United States. Researchers believe that nvCJD is likely caused from an exposure to bovine spongiform encephalopathy (BSE), a fatal brain disease in cattle. Exposure occurs through ingestion of beef products from infected cows. In fact, all reported cases of nvCJD have occurred in nations in which BSE likewise occurred.
been reported in Canada, Ireland, Italy, and the United States. Researchers believe that nvCJD is likely caused from an exposure to bovine spongiform encephalopathy (BSE), a fatal brain disease in cattle. Exposure occurs through ingestion of beef products from infected cows. In fact, all reported cases of nvCJD have occurred in nations in which BSE likewise occurred.
In an attempt to prevent BSE from entering the United States, the importing of cattle, sheep, and goats from European countries has been restricted. The Centers for Disease Control and Prevention (CDC) and the World Health Organization continue to study CJD and nvCJD.
Causes
The organism that causes CJD is difficult to identify because no foreign ribonucleic acid or deoxyribonucleic acid have been linked to this disease. CJD may be caused by a specific protein called a prion, which lacks nucleic acids, resists proteolytic digestion, and spontaneously aggregates in the brain. Most cases of conventional CJD are sporadic; 5% to 15% are familial, with an autosomal dominant pattern of inheritance. Although CJD isn’t transmitted by casual contact, person-to-person transmission can occur as a result of specific medical procedures, such as corneal and cadaveric dura mater grafts. Isolated cases are attributed to treatment during childhood with human growth hormone and to improperly decontaminated neurosurgical instruments and brain electrodes.
