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03-22-2008, 01:26 PM | #8 | ||
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Original Paper
Association between Deposition of Beta-Amyloid and Pathological Prion Protein in Sporadic Creutzfeldt-Jakob Disease Laura Debatina, Johannes Strefferb, Markus Geissenc, Jakob Matschkec, Adriano Aguzzia, Markus Glatzela, c aInstitute of Neuropathology, and bDivision of Psychiatry Research, University Hospital Zurich, Zurich, Switzerland; cInstitute of Neuropathology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany Address of Corresponding Author Neurodegenerative Dis (DOI: 10.1159/000121389) ---------------------------------------------------------------------------- ---- Key Words Sporadic Creutzfeldt-Jakob disease Alzheimer's disease Deposition of -amyloid Prion protein ---------------------------------------------------------------------------- ---- Abstract Background: Alzheimer's disease (AD) and prion diseases such as sporadic Creutzfeldt-Jakob disease (sCJD) share common features concerning their molecular pathogenesis and neuropathological presentation and the coexistence of AD and CJD in patients suggest an association between the deposition of the proteolytically processed form of the amyloid precursor protein, -amyloid (A), which deposits in AD, and the abnormal form of the prion protein, PrPSc, which deposits in sCJD. Methods: We have characterized sCJD patients (n = 14), AD patients (n = 5) and nondemented controls (n = 5) with respect to the deposition of PrPSc and A morphologically, biochemically and genetically and correlated these findings to clinical data. Results: sCJD-diseased individuals with abundant deposits of A present with a specific clinicopathological profile, defined by higher age at disease onset, long disease duration, a genetic profile and only minimal amounts of PrPSc in the cerebellum. Conclusion: The co-occurrence of pathological changes typical for sCJD and AD in combination with the inverse association between accumulation of A and PrPSc in a subgroup of sCJD patients is indicative of common pathways involved in the generation or clearance of A and PrPSc in a subgroup of sCJD patients. Copyright © 2008 S. Karger AG, Basel ---------------------------------------------------------------------------- ---- Author Contacts Markus Glatzel Institute of Neuropathology, University Medical Center Hamburg-Eppendorf Martinistrasse 52, DE-20246 Hamburg (Germany) Tel. +49 40 42 803 2218, Fax +49 40 42 803 4929 E-Mail m.glatzel@uke.uni-hamburg.de http://content.karger.com/produktedb...file=000121389 Singeltary, Sr et al. JAMA.2001; 285: 733-734. Diagnosis and Reporting of Creutzfeldt-Jakob Disease Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally. Terry S. Singeltary, Sr Bacliff, Tex 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT http://jama.ama-assn.org/cgi/content...urcetype=HWCIT see my full text on Alzheimers and CJD blog here ; http://betaamyloidcjd.blogspot.com/2...n-of-beta.html TSS |
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