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The first 300 words of the full text of this section appear below.

Section 3. Summaries of Infectious Diseases

Prion Diseases

Transmissible Spongiform Encephalopathies 194

Clinical Manifestations
Etiology
Epidemiology
Diagnostic Tests
Treatment
Isolation of the Hospitalized Patient
Control Measures

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CLINICAL MANIFESTATIONS

Transmissible spongiform encephalopathies (TSEs), or prion diseases, constitute a group of rare, rapidly progressive, universally fatal neurodegenerative syndromes of humans and animals that are characterized by neuronal degeneration, spongiform change, gliosis, and accumulation of an abnormal protease-resistant amyloid protein (protease-resistant prion protein [PrPres], scrapie prion protein [PrPsc], or as suggested by the World Health Organization [WHO], TSE-associated PrP [PrPTSE]) distributed diffusely throughout the brain and sometimes also in discrete plaques.

Human TSEs include several diseases: Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease, fatal familial and sporadic insomnia syndromes, kuru, and variant CJD (vCJD, or mad cow disease). Classic CJD can be sporadic (approximately 85% of cases), familial (approximately 15% of cases), or iatrogenic (fewer than 1% of cases). Sporadic CJD most commonly is a disease of the elderly (median age of death, 68 years in the United States) but also rarely has been described in adolescents older than 13 years of age and young adults. Iatrogenic CJD has been acquired through injection of cadaveric pituitary hormones (growth hormone and human gonadotropin), dura mater allografts, corneal transplantation, and instrumentation of the brain at neurosurgery or depth-electrode electroencephalographic recording. In 1996, an outbreak of vCJD linked to exposure to tissues from bovine spongiform encephalopathy (BSE)-infected cattle was reported in the United Kingdom. Since the end of 2003, 3 presumptive cases of transfusion-transmitted vCJD have been reported as well as 1 probable transfusion-transmitted preclinical vCJD infection. The best-known TSEs affecting animals are scrapie of sheep, BSE, and a chronic wasting disease of North American deer, elk, and moose. Except for vCJD, thought to have originated from BSE, no other human TSE has been attributed . . . [Go to Full Text]


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