According to a story from ABC News, Tony Gibson of Tennessee was recently diagnosed with the human form of mad cow disease, which is also known as Creutzfeldt-Jakob disease (CJD). He first began to experience symptoms of the illness around a year ago. He was just 32 years old. In years past, mad cow disease made headlines because of the fear that it could easily spread to humans. However, these fears were pretty exaggerated, as the disease only affects about 500 people in the US. Regardless, there is some valuable information that you should now about this unusual illness.
Creutzfeldt-Jakob disease is caused by the misfolding of proteins called prions in the brain. Prions are implicated in a number of fatal neurodegenerative diseases. The misfolded prions are believed to affect signaling processes in the brain. What triggers this process of misfolding to begin is not known in many cases. In a small number of cases, the disease is familial, being caused by genetic mutations. Mutations are linked to around 15 percent of cases. The disease can also be transmitted by eating contaminated meat or exposure to nervous system tissue. It cannot be transmitted by mere physical contact between a patient and a healthy person.
Creutzfeldt-Jakob Disease and Mad Cow Disease
The reason Creutzfeldt-Jakob disease is related to mad cow disease, more scientifically known as bovine spongiform encephalopathy, is because the disease mechanism is essentially identical. It is also possible, although extremely rare, to get a different type of Creutzfeldt-Jakob disease, called variant Creutzfeldt-Jakob disease, by eating beef from an animal affected by mad cow. This happens only very rarely.
This disease generally causes subtle symptoms at first, but then begins to progress rapidly. Symptoms include personality and mood changes, trouble with cognition, and memory loss. Unusual, jerking movements, loss of coordination, depression, anxiety, and difficulty walking also can develop. The symptoms ultimately begin to resemble fast progressing dementia. Patients eventually enter a coma.
There is no cure for Creutzfeldt-Jakob disease. The are no known methods for altering the progression of the disease, and treatment is mostly supportive. 90 percent of patients die within one year of diagnosis, and it can be fatal in as little as a few weeks or months. There is a dire need for more effective treatments for Creutzfeldt-Jakob disease.