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Previous Volume 337:1821-1828 December 18, 1997 Number 25 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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The transmissible spongiform encephalopathies are caused by infectious agents but usually present as genetic or sporadic disorders. The nature of the infectious agents is not known. In 1986 a new transmissible spongiform encephalopathy, which was called bovine spongiform encephalopathy or "mad cow disease," was described in Britain.¹ In 1996 the British noted cases of Creutzfeldt–Jakob disease that differed from other cases of the same disease and named this entity new variant Creutzfeldt–Jakob disease.² The possible association of new variant Creutzfeldt–Jakob disease with bovine spongiform encephalopathy brought these rare diseases to public and political attention. This review summarizes the background information . . . [Full Text of this Article]

Biology of the Transmissible Spongiform Encephalopathies

Characteristics of the Infectious Agents

Pathogenesis and Loci of Infection

Species Barrier

Strains

Host Genes That Influence Incubation Periods

Chemistry and Molecular Biology of Transmissible Spongiform Encephalopathies

PrP

Neuropathology of Transmissible Spongiform Encephalopathies

Transmissible Spongiform Encephalopathies in Animals

Bovine Spongiform Encephalopathy (Mad Cow Disease)

Human Transmissible Spongiform Encephalopathies

Kuru

Genetics of PrP, Familial Creutzfeldt–Jakob Disease, Gerstmann–Sträussler–Scheinker Disease, and Fatal Familial Insomnia

Creutzfeldt–Jakob Disease

            Iatrogenic Creutzfeldt–Jakob Disease

            Sporadic Creutzfeldt–Jakob Disease

            New Variant Creutzfeldt–Jakob Disease

Conclusions

Source Information

From the Departments of Pediatrics and Microbiology, University of Rochester Medical Center, Rochester, NY 14642-8777, where reprint requests should be addressed to Dr. Haywood.

References

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• Favereaux, A., Quadrio, I., Vital, C., Perret-Liaudet, A., Anne, O., Laplanche, J.-L., Petry, K. G., Vital, A. (2004). Pathologic Prion Protein Spreading in the Peripheral Nervous System of a Patient With Sporadic Creutzfeldt-Jakob Disease. Arch Neurol 61: 747-750 [Abstract] [Full Text]  
• Ochel, H.-J., Gademann, G., Trepel, J., Neckers, L. (2003). Modulation of prion protein structural integrity by geldanamycin. Glycobiology 13: 655-660 [Abstract] [Full Text]  
• Zanusso, G., Ferrari, S., Cardone, F., Zampieri, P., Gelati, M., Fiorini, M., Farinazzo, A., Gardiman, M., Cavallaro, T., Bentivoglio, M., Righetti, P. G., Pocchiari, M., Rizzuto, N., Monaco, S. (2003). Detection of Pathologic Prion Protein in the Olfactory Epithelium in Sporadic Creutzfeldt-Jakob Disease. NEJM 348: 711-719 [Abstract] [Full Text]  
• 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 Text]  
• Johnson, R. T., Gibbs, C. J. (1998). Creutzfeldt-Jakob Disease and Related Transmissible Spongiform Encephalopathies. NEJM 339: 1994-2004 [Full Text]