Variant Creutzfeldt-Jakob disease, or vCJD, is a rare degenerative brain disorder that leads to dementia and eventual death. Scientists believe an abnormal protein known as a prion causes vCJD by causing other proteins within the body to collapse abnormally, decreasing their ability to function.
History
The first person to develop vCJD became ill in January 1994 in the United Kingdom, according to the World Health Organization. The WHO goes on to explain that between January 1994 and November 2002, only 129 cases of vCJD had been reported in total, including 93 confirmed cases, 24 probable cases and 12 where vCJD was strongly suspected but could not be confirmed.
Transmission
Variant CJD is strongly linked to a disease that affects cattle known as bovine spongiform encephalopathy, or "mad cow" disease, a disease first reported in the United Kingdom in 1986. The Centers for Disease Control and Prevention explains that strong evidence suggests that the prion that causes bovine spongiform encephalopathy is the same prion that causes vCJD. The most likely exposure to humans occurred through bovine-based food such as infected beef.
Effects
MayoClinic.com states that vCJD belongs to a group of diseases known as transmissible spongiform encephalopathy. The word spongiform refers to the spongy holes visible in the affected brain tissue under a microscope. Over time, the brain tissues deteriorate with the patient's symptoms becoming more and more severe.
Symptoms
Patients with vCJD will often present with painful muscle twitches as well as prominent psychiatric and behavioral symptoms. These symptoms can include dementia, delirium, speech impairment, profound confusion, sleepiness, blurred vision and nervousness. Changes in gait, muscle twitches, lack of coordination, muscle stiffness and seizures are also common symptoms, according to the University of Maryland Medical Center.
Prognosis
The prognosis for vCJD is poor. MayoClinic.com warns that within six months of symptom presentation a person will become incapable of caring for himself. The disease is fatal, with death occurring usually within 14 to 24 months after diagnosis. The cause of death is commonly heart failure, respiratory failure or infection.
