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What is Creutzfeldt-Jakob Disease?

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  • Written By: Mary McMahon
  • Edited By: Bronwyn Harris
  • Last Modified Date: 19 November 2016
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Creutzfeldt-Jakob Disease is a degenerative nervous system disorder which ultimately attacks the brain, causing death with seven months to two years of the appearance of symptoms. Currently, there is no cure for Creutzfeldt-Jakob Disease, although medications can be used to treat the symptoms and make the patient comfortable. Like other diseases of the nervous system, Creutzfeldt-Jakob Disease causes irregular movements, difficulty in controlling the muscles, inability to perform tasks requiring fine motor control, and dementia as the brain finally succumbs. It was first identified in the 1920s by a pair of German scientists, although the cause was imperfectly understood until further study was undertaken later in the twentieth century.

Many people had not heard of Creutzfeldt-Jakob Disease until an outbreak of a related disease, Bovine Spongiform Encephalopathy (BSE). Both Creutzfeldt-Jakob Disease and BSE are Transmissible Spongiform Encephalophathies (TSEs), and they are caused by rogue prions. A prion is a type of protein; most prions are harmless, but when one becomes misfolded, it can infect other proteins, causing them to twist and clump together in formations called plaques. These plaques interfere with nervous system function, and in the brain the alteration of the normal cellular arrangement causes holes to appear in the brain tissue, leading to a sponge like appearance.

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There are three types of Creutzfeldt-Jakob Disease: sporadic, inherited, and acquired. Sporadic Creutzfeldt-Jakob Disease is the culprit behind 85% of all cases, and appears randomly due to a mutation in the prions of the body. Inherited Creutzfeldt-Jakob Disease occurs because of a faulty gene, which causes the formation of harmful prions, and accounts for five to 10% of Creutzfeldt-Jakob Disease cases. The acquired form of the disease comes from contact with infectious brain and nervous system tissues. When BSE crosses species and infects humans, it is called Variant Creutzfeldt-Jakob Disease, but this accounts for a relatively small number of cases.

Once symptoms begin to manifest, onset of the disease is rapid and unstoppable. Patients may take pain medication to help manage physical symptoms, along with antispasmodics to help control their muscles. True diagnosis is also impossible, as it requires an autopsy of the brain. However, the symptoms are well described in medical literature, and when neurologists deal with patients manifesting the symptoms of Creutzfeldt-Jakob Disease, they operate under the assumption that this is the cause of the neurological disorder after ruling out other potential causes.

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