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Appendices

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Section 3. Summaries of Infectious Diseases

PRION DISEASES

Transmissible Spongiform Encephalopathies 1

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] or scrapie prion protein [PrPsc]) distributed diffusely throughout the brain and sometimes also in discrete plaques. Pathologic involvement of other organ systems has been reported in animal TSEs, but not in humans.

The human TSEs include several diseases: Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease, fatal familial and sporadic insomnia syndromes, kuru, and variant CJD (vCJD). Classic CJD can be sporadic (approximately 85% of cases), familial (approximately 15%), or iatrogenic (<1%). Sporadic CJD has been described in adolescents older than 13 years of age and in 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. Cases of transfusion-transmitted vCJD have been reported. In 1996, an outbreak of vCJD linked to exposure to tissues from bovine spongiform encephalopathy (BSE)-infected cattle was reported in the United Kingdom. The best-known TSEs affecting animals are scrapie of sheep, BSE, and a chronic wasting disease of North American deer and elk.

Creutzfeldt-Jakob disease manifests as a dementing syndrome with progressive defects in memory, personality, and other higher cortical functions. At presentation, approximately one third of patients have cerebellar dysfunction, including ataxia and dysarthria. Iatrogenic CJD may manifest as dementia (as in dural allograft transplant recipients) or as cerebellar signs (as observed in virtually all people with peripherally . . . [Go to Full Text]


Related text in Red Book:

Transfusion-Transmitted Agents: Known Threats and Potential Pathogens

Red Book 2006: 113-121. [Extract] [Full Version]  



This topic has been referenced by these articles:

  • Whitley, R. J., MacDonald, N., Asher, D. M., the Committee on Infectious Diseases, (2000). Technical Report: Transmissible Spongiform Encephalopathies: A Review for Pediatricians. Pediatrics 106: 1160-1165 [Abstract] [Full Version]