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Review Article
Medical Progress
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Volume 339:1994-2004 December 31, 1998 Number 27
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Creutzfeldt–Jakob Disease and Related Transmissible Spongiform Encephalopathies
Richard T. Johnson, M.D., and Clarence J. Gibbs, Ph.D.

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Until 30 years ago, Creutzfeldt–Jakob disease was an obscure form of dementia unknown to most physicians. The name is now familiar to the medical community as the major transmissible spongiform encephalopathy (or prion disease) in humans and to research scientists because of its strange causative agent with novel modes of replication and transmission. Furthermore, the term "Creutzfeldt–Jakob disease" has caused distress to patients who have been notified that they may have received tainted blood or blood products, and it has been popularized by extensive media coverage of bovine spongiform encephalopathy ("mad cow disease"), with its profound economic impact in Europe . . . [Full Text of this Article]

Transmissible Spongiform Encephalopathies

Nature of Prions

Pathogenesis

Sporadic Creutzfeldt–Jakob Disease

Early Recognition and Epidemiology

Clinical Disease

Laboratory Findings

Pathological Findings

Differential Diagnosis

Risks of Infection from Patients and Tissues

Iatrogenic Creutzfeldt–Jakob Disease

Surgical Transmission

Transmission by Pituitary Hormones

Risks Associated with Blood Products

Familial Creutzfeldt–Jakob Disease

Distinctive Syndromes

Genetic Counseling

Bovine Spongiform Encephalopathy and New-Variant Creutzfeldt–Jakob Disease

The Mad-Cow Outbreak

New-Variant Creutzfeldt–Jakob Disease

Issues of Concern in North America

Conclusions


Source Information

From the Department of Neurology, Johns Hopkins University School of Medicine (R.T.J., C.J.G.), and the Department of Molecular Microbiology and Immunology, Johns Hopkins University School of Hygiene and Public Health (R.T.J.), Baltimore; the National Neuroscience Institute of Singapore, Singapore (R.T.J.); and the Laboratory of Central Nervous System Studies, National Institutes of Health, Bethesda, Md. (C.J.G.).

Address reprint requests to Dr. Johnson at Meyer 6-181, Johns Hopkins Hospital, Baltimore, MD 21287.

References


Related Letters:

Creutzfeldt–Jakob Disease
Hansen M., Gray R. H., Cavanagh H. D., Hogan R. N., Johnson R. T., Gibbs C. J.
Extract | Full Text  
N Engl J Med 1999; 340:1687-1689, May 27, 1999. Correspondence

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