Creutzfeldt-Jakob disease (CJD)
A degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90% of patients die within a year. In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. There are three major categories of CJD: {{}}Sporadic CJD: {{}}In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85% of cases. Hereditary CJD: In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10% of cases of CJD in the United States are hereditary. Acquired CJD: In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. Since CJD was first described in 1920, fewer than 1% of cases have been acquired CJD. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. Fatal familial insomnia and GSS are extremely rare hereditary diseases, found in just a few families around the world. Other TSEs are found in specific kinds of animals. These include bovine spongiform encephalopathy (BSE), which is found in cows and often referred to as "mad cow" disease, scrapie, which affects sheep and goats, mink encephalopathy, and feline encephalopathy. Similar diseases including chronic wasting disease (CWD) occur in elk, deer, and exotic zoo animals. Symptoms: {{}}CJD is a rapidly progressive dementia. Initially, patients experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. People with the disease also may experience insomnia, depression, or unusual sensations. CJD does not cause a fever or other flu-like symptoms. As the illness progresses, the patients' mental impairment becomes severe. They often develop involuntary muscle jerks called myoclonus, and they may go blind. They eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these patients and can lead to death.

Medical dictionary. 2011.

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  • Creutzfeldt–Jakob disease — Classification and external resources Tonsil biopsy in variant CJD. Prion Protein immunostaining. ICD 10 A …   Wikipedia

  • Creutzfeldt-Jakob disease — Infobox Disease Name = Creutzfeldt Jakob disease Caption = DiseasesDB = 3166 ICD10 = ICD10|A|81|0|a|80, ICD10|F|02|1|f|00 ICD9 = ICD9|046.1 ICDO = OMIM = 123400 MedlinePlus = eMedicineSubj = neuro eMedicineTopic = 725 MeshID = D007562 Creutzfeldt …   Wikipedia

  • Creutzfeldt-Jakob disease — CJD a rapidly progressive rare neurological disease, a form of human spongiform encephalopathy in which dementia progresses to death after a period of 3–12 months. There is no effective treatment. The causative agent is an abnormal prion protein… …   The new mediacal dictionary

  • Creutzfeldt-Jakob disease — noun rare (usually fatal) brain disease (usually in middle age) caused by an unidentified slow virus; characterized by progressive dementia and gradual loss of muscle control • Syn: ↑CJD, ↑Jakob Creutzfeldt disease • Hypernyms: ↑brain disorder,… …   Useful english dictionary

  • Creutzfeldt-Jakob disease — Creutz·feldt Ja·kob disease also Creutz·feld Ja·kob disease .krȯits .felt .yä (.)kōb n a rare progressive fatal spongiform encephalopathy now usu. considered to be caused by a prion and marked by the development of porous brain tissue, premature …   Medical dictionary

  • Creutzfeldt-Jakob disease — /kroyts felt yah kawp/, Pathol. a rare, usually fatal brain disorder caused by an unidentified pathogen and characterized by progressive dementia, blindness, and involuntary movements. Also, Creutzfeldt Jacob disease. Also called Jakob… …   Universalium

  • Creutzfeldt-Jakob disease — Creutz·feldt Ja·kob dis·ease || ‚krɔɪtsfelt jækÉ’b CJD, rare degenerative brain disease named after Hans G. Creutsfeldt and Alfons Jakob (disease is characterized by memory loss, jerky movements, stiff posture and seizures caused by a… …   English contemporary dictionary

  • Creutzfeldt-Jakob disease — Creutz|feldt Ja|kob dis|ease [ ,krɔıtsfelt jakɔb dı,ziz ] noun uncount the disease CJD …   Usage of the words and phrases in modern English

  • Creutzfeldt-Jakob disease — UK [ˌkrɔɪtsfelt ˈjækɒb dɪˌziːz] / US [ˌkrɔɪtsfelt ˈjɑkɔb dɪˌzɪz] noun [uncountable] medical the disease CJD …   English dictionary

  • Creutzfeldt-Jakob disease — /krɔɪtsfɛlt ˈjakəp dəziz/ (say kroytsfelt yahkuhp duhzeez) noun a rare, fatal brain disease caused by a slow virus and characterised by increasing dementia, impaired and disturbed vision, twitching and loss of control of physical functions.… …   Australian English dictionary

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