Creutzfeldt-Jakob Disease (CJD) is a rare, devastating, degenerative, and mostly fatal brain disorder. It is part of a group of diseases called prion diseases that occurs in humans and certain animals. The leading scientific theory at this time maintains that CJD is caused by a type of protein called a prion.
This group of diseases first came to the public attention in the mid-1980s as a result of the outbreak of bovine spongioform encephalopathy (BSE) in the United Kingdom. BSE is a prion disease in cattle. Tissue from infected animals may have been used in animal feed, leading to the spread of the disease and growing concern over whether this disease could pass to humans.
In humans, the most widely known of the prion diseases is CJD, which affects around one person per million per year. In the United States, around 250 to 300 new cases are reported per year. There are three major categories of CJD: inherited, sporadic, and acquired. These categories mainly refer to the way the disease was acquired rather than the symptoms that accompany the disease. One of the variant types of acquired CJD observed in younger populations (ages 16–39), variant Creutzfeldt-Jakob disease (vCJD), has been associated with the consumption of beef and beef products from animals infected with BSE. In an animal infected with BSE, prions are concentrated in two areas of the body: the nervous system tissues, including the brain, spinal cord, and eyes, and the lymphatic tissues, such as the lymph nodes, bone marrow, and spleen. The greatest risk according to the Mayo Clinic for acquiring vCJD is associated with ingesting these tissues from BSE-infected cattle. At this time, there is no evidence that milk products from a BSE-infected animal pose a risk to humans. However, according to the Centers for Disease Control and Prevention, there is no precise method for determining the risk for acquiring this variant form of CJD from eating beef and beef products from cattle in countries that have reported confirmed cases of BSE.
There is no single diagnostic test for the diagnosis of CJD at this time. The only way to conclusively diagnose CJD is through a brain biopsy or autopsy. A brain biopsy is a highly invasive procedure and involves removing a small piece of tissue from the patient’s brain so that it can be examined by a neurologist. Due to the invasive nature of this procedure coupled with the fact that a correct diagnosis of CJD does not help in the treatment or prognosis of the disease, a brain biopsy is discouraged. When autopsies have been performed on individuals that suffered from CJD, the brain tissue looks “spongy” because it is punctured by many tiny holes where cells have been damaged.
With CJD, typically the symptoms begin between ages 45 and 75. In the early stages of the disease, patients may have significant memory problems and may demonstrate changes in their behavior, lack of coordination, and visual disturbances. As the illness progresses, mental deterioration becomes the primary symptom, with observed involuntary movements, blindness, weakness of the extremities, and possibly coma. As mentioned above, this disease is fatal and about 90% of patients die within 1 year.
What can be done for individuals suffering from CJD? Currently there is no treatment or medication that can cure CJD. Most treatments are aimed at alleviating the symptoms experienced and helping the patient feel as comfortable and pain free as possible. Opiate drugs are frequently used because they can help relieve the pain, and the drugs clonazepam and sodium valproate may help relieve involuntary muscle jerks.
In conclusion, CJD is a very potent and fatal brain disorder thought to be caused by proteins called prions. It affects mainly individuals between 45 and 75 years of age, but the newest variant of the disease (vCJD) has been observed in younger individuals (ages 16–39). There is no specific procedure to diagnose CJD other than a brain biopsy or autopsy. Since this disease is not curable, treatments center around making the patient more comfortable and reducing the pain.
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- Mayo Foundation for Medical Education and (2004, May 18). Creutzfeldt-Jakob disease. Retrieved from http:// www.mayoclinic.com/invoke.cfm?id=DS00531
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