Until 30 years ago, CreutzfeldtJakob disease was an obscureform of dementia unknown to most physicians. The name is nowfamiliar to the medical community as the major transmissiblespongiform encephalopathy (or prion disease) in humans and toresearch scientists because of its strange causative agent withnovel modes of replication and transmission. Furthermore, theterm "CreutzfeldtJakob disease" has caused distress topatients who have been notified that they may have receivedtainted blood or blood products, and it has been popularizedby 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 CreutzfeldtJakob Disease
Early Recognition and Epidemiology
Clinical Disease
Laboratory Findings
Pathological Findings
Differential Diagnosis
Risks of Infection from Patients and Tissues
Iatrogenic CreutzfeldtJakob Disease
Surgical Transmission
Transmission by Pituitary Hormones
Risks Associated with Blood Products
Familial CreutzfeldtJakob Disease
Distinctive Syndromes
Genetic Counseling
Bovine Spongiform Encephalopathy and New-Variant CreutzfeldtJakob Disease
The Mad-Cow Outbreak
New-Variant CreutzfeldtJakob 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:
CreutzfeldtJakob 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
This article has been cited by other articles:
Fulbright, R.K., Hoffmann, C., Lee, H., Pozamantir, A., Chapman, J., Prohovnik, I.
(2008). MR Imaging of Familial Creutzfeldt-Jakob Disease: A Blinded and Controlled Study. Am. J. Neuroradiol.
29: 1638-1643
[Abstract][Full Text]
Herczenik, E., Gebbink, M. F. B. G.
(2008). Molecular and cellular aspects of protein misfolding and disease. FASEB J.
22: 2115-2133
[Abstract][Full Text]
Kashkevich, K., Humeny, A., Ziegler, U., Groschup, M. H., Nicken, P., Leeb, T., Fischer, C., Becker, C.-M., Schiebel, K.
(2007). Functional relevance of DNA polymorphisms within the promoter region of the prion protein gene and their association to BSE infection. FASEB J.
21: 1547-1555
[Abstract][Full Text]
Chang, B., Cheng, X., Yin, S., Pan, T., Zhang, H., Wong, P., Kang, S.-C., Xiao, F., Yan, H., Li, C., Wolfe, L. L., Miller, M. W., Wisniewski, T., Greene, M. I., Sy, M.-S.
(2007). Test for Detection of Disease-Associated Prion Aggregate in the Blood of Infected but Asymptomatic Animals. CVI
14: 36-43
[Abstract][Full Text]
Ukisu, R., Kushihashi, T., Tanaka, E., Baba, M., Usui, N., Fujisawa, H., Takenaka, H.
(2006). Diffusion-weighted MR Imaging of Early-Stage Creutzfeldt-Jakob Disease: Typical and Atypical Manifestations. RadioGraphics
26: S191-S204
[Abstract][Full Text]
Johnson, R. T., Gonzalez, R. G., Frosch, M. P.
(2005). Case 27-2005 - An 80-Year-Old Man with Fatigue, Unsteady Gait, and Confusion. NEJM
353: 1042-1050
[Full Text]
Ukisu, R., Kushihashi, T., Kitanosono, T., Fujisawa, H., Takenaka, H., Ohgiya, Y., Gokan, T., Munechika, H.
(2005). Serial Diffusion-Weighted MRI of Creutzfeldt-Jakob Disease. Am. J. Roentgenol.
184: 560-566
[Abstract][Full Text]
Hegglin, A., Munzer, T.
(2005). RAPID NEUROLOGICAL DECLINE AND DEATH IN A 79-YEAR-OLD WOMAN. J. Gerontol. A Biol. Sci. Med. Sci.
60: 133-134
[Full Text]
Shapiro, J. M., Shujaat, A., Wang, J., Chen, X.
(2004). Creutzfeldt-Jakob Disease Presenting as Refractory Nonconvulsive Status Epilepticus. J Intensive Care Med
19: 345-348
[Abstract]
Russelakis-Carneiro, M., Hetz, C., Maundrell, K., Soto, C.
(2004). Prion Replication Alters the Distribution of Synaptophysin and Caveolin 1 in Neuronal Lipid Rafts. Am. J. Pathol.
165: 1839-1848
[Abstract][Full Text]
Rentz, C. A.
(2003). Creutzfeldt-Jakob disease: Two case studies. AM J ALZHEIMERS DIS OTHER DEMEN
18: 171-180
[Abstract]
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]
Doherty, C P, Schlossmacher, M, Torres, N, Bromfield, E, Samuels, M A, Folkerth, R
(2002). Hashimoto's encephalopathy mimicking Creutzfeldt-Jakob disease: brain biopsy findings. J. Neurol. Neurosurg. Psychiatry
73: 601-602
[Full Text]
Mao-Draayer, Y., Braff, S. P., Nagle, K. J., Pendlebury, W., Penar, P. L., Shapiro, R. E.
(2002). Emerging Patterns of Diffusion-Weighted MR Imaging in Creutzfeldt-Jakob Disease: Case Report and Review of the Literature. Am. J. Neuroradiol.
23: 550-556
[Abstract][Full Text]
Filla, A., De Michele, G., Cocozza, S., Patrignani, A., Volpe, G., Castaldo, I., Ruggiero, G., Bonavita, V., Masters, C., Casari, G., Bruni, A.
(2002). Early onset autosomal dominant dementia with ataxia, extrapyramidal features, and epilepsy. Neurology
58: 922-928
[Abstract][Full Text]
Prusiner, S. B.
(2001). Neurodegenerative Diseases and Prions. NEJM
344: 1516-1526
[Full Text]
Roos, R. P.
(2001). Controlling New Prion Diseases. NEJM
344: 1548-1551
[Full Text]
Kennedy, R. H., Hogan, R. N., Brown, P., Holland, E., Johnson, R. T., Stark, W., Sugar, J.
(2001). Eye Banking and Screening for Creutzfeldt-Jakob Disease. Arch Ophthalmol
119: 721-726
[Abstract][Full Text]
Hannah, E. L., Belay, E. D., Gambetti, P., Krause, G., Parchi, P., Capellari, S., Hoffman, R. E., Schonberger, L. B.
(2001). Creutzfeldt-Jakob disease after receipt of a previously unimplicated brand of dura mater graft. Neurology
56: 1080-1083
[Abstract][Full Text]
Liu, T., Li, R., Wong, B.-S., Liu, D., Pan, T., Petersen, R. B., Gambetti, P., Sy, M.-S.
(2001). Normal Cellular Prior Protein Is Preferentially Expressed on Subpopulations of Murine Hemopoietic Cells. J. Immunol.
166: 3733-3742
[Abstract][Full Text]
Schaefer, P. W., Grant, P. E., Gonzalez, R. G.
(2000). Diffusion-weighted MR Imaging of the Brain. Radiology
217: 331-345
[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]
Lemstra, A. W., van Meegen, M. T., Vreyling, J.P, Meijerink, P. H. S., Jansen, G. H., Bulk, S., Baas, F., van Gool, W. A.
(2000). 14-3-3 testing in diagnosing Creutzfeldt-Jakob disease: A prospective study in 112 patients. Neurology
55: 514-516
[Abstract][Full Text]
Vrancken, A. F. J. E., Frijns, C. J. M., Ramos, L. M. P.
(2000). FLAIR MRI in sporadic Creutzfeldt-Jakob disease. Neurology
55: 147-148
[Full Text]
Tagliati, M., Perl, D. P, Drayer, B., Olanow, C W.
(2000). Progressive dementia and gait disorder in a 78 year old woman. J. Neurol. Neurosurg. Psychiatry
68: 526-531
[Full Text]
Poser, S., Mollenhauer, B., Krau{beta}, A., Zerr, I., Steinhoff, B. J., Schroeter, A., Finkenstaedt, M., Schulz-Schaeffer, W. J., Kretzschmar, H. A., Felgenhauer, K.
(1999). How to improve the clinical diagnosis of Creutzfeldt-Jakob disease. Brain
122: 2345-2351
[Abstract][Full Text]
Frigg, R., Klein, M. A., Hegyi, I., Zinkernagel, R. M., Aguzzi, A.
(1999). Scrapie Pathogenesis in Subclinically Infected B-Cell-Deficient Mice. J. Virol.
73: 9584-9588
[Abstract][Full Text]
Martin, J. B.
(1999). Molecular Basis of the Neurodegenerative Disorders. NEJM
340: 1970-1980
[Full Text]
Sanders, T A B
(1999). Food production and food safety. BMJ
318: 1689-1693
[Full Text]
Hansen, M., Gray, R. H., Cavanagh, H. D., Hogan, R. N., Johnson, R. T., Gibbs, C. J.
(1999). Creutzfeldt-Jakob Disease. NEJM
340: 1687-1689
[Full Text]
Kim, S. J., Rahbar, R., Hegde, R. S.
(2001). Combinatorial Control of Prion Protein Biogenesis by the Signal Sequence and Transmembrane Domain. J. Biol. Chem.
276: 26132-26140
[Abstract][Full Text]