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A form of neurodegenerative human prion (acronym for “proteinaceous infectious particles”) disorder, which belongs to a group of diseases known as transmissible spongiform encephalopathies.

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Worldwide, the incidence of Creutzfeldt-Jakob disease (CJD) in the general population is approximately 1:1,000,000 per year. A variant of CJD (vCJD) has been associated with bovine spongiform encephalopathy (BSE) and has the highest occurrence in the United Kingdom. As of November 2005, the total number of definite or probable vCJD cases (dead and alive) in the United Kingdom was 158 (in 108 cases with neuropathologic confirmation). A causal association between vCJD and BSE is supported by epidemiologic and laboratory evidence in areas with high prevalence of BSE and the lack thereof in basically BSE-free regions.

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The occurrence of CJD, like other forms of prion diseases, is familial (5-10% of cases, with autosomal dominant inheritance caused by mutations in the PRNP [prion protein] gene, which has been mapped to 20pter-p12), sporadic (approximately 90% of patients with noninherited mutation of the prion protein), or infectious (iatrogenic with the first reported case of vCJD in 1974). The infectious agent in prion disease consists almost entirely of a host-encoded protease-resistant, neuronal cell surface sialoglycoprotein termed prion protein.

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The cause is not fully understood but is believed to be related to misfolding of the normal endogenous cellular prion protein PrPC into a new pathogenic conformation PrPSc encoded by PRNP. Prion protein is necessary for normal synaptic function. Iatrogenic and infectious transmission has occurred via tissue transplants (e.g., corneal transplants and no longer used dura mater grafts), cadaveric pituitary gland extracts (e.g., for human growth hormone [hGH] and gonadotropin production, now replaced by synthetic hormones), and contamination by neurosurgical instruments and stereotactic electroencephalography electrodes. Currently, blood is considered to pose a theoretical risk only, and no cases related to blood transmission have been reported. Accumulation of PrPSc in the central nervous system of affected individuals is responsible for the typical degenerative changes, such as loss of neurons, vacuolization, and finally death.

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There is no definitive clinical diagnostic test apart from the neuropathologic investigation. Patients have variable clinical and pathologic pictures. Pathology findings include diffuse nerve cell degeneration, status spongiosus, and glial proliferation. The electroencephalogram may show characteristic periodic complex changes.

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The vast majority of patients are between 50 and 70 years old at the onset of the disease, although significantly younger affected patients have been reported. The onset of familial cases is significantly earlier than in other forms. The course of the illness may last from a few weeks to several years, although in general it is rapidly progressive. It is uniformly fatal, with the average length of survival from onset of the disease being approximately 6 months. Affected patients present with signs of dementia that often are accompanied by behavioral changes and psychiatric anomalies. Characteristic electroencephalographic changes can be detected (a slow background rhythm with superimposed periodic biphasic or triphasic ...

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