Creutzfeldt–Jakob disease in Germany: a prospective 12-year surveillance
1National TSE Reference Center at Department of Neurology, Georg-August University Göttingen, Germany, 2Department of Neuroradiology, Georg-August University Göttingen, Germany, 3Department of Neuropathology, Georg-August University Göttingen, Germany, 4Epilepsy Center Kork, Diakonie Kork, Germany and 5Department of Neuropathology, Ludwig-Maximilian University Munich, Germany
Correspondence to: Inga Zerr, MD, National Reference Center for TSE, Department of Neurology, Georg-August University Göttingen, Robert-Koch-Strasse 40, 37075 Göttingen, Germany E-mail: IngaZerr{at}med.uni-goettingen.de
Creutzfeldt–Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a worldwide incidence of 1–1.5 per million. As in other countries, a CJD surveillance unit with a clinical and neuropathological approach was established in Goettingen (Germany) in 1993. Here we report the epidemiological data from a prospective 12-year surveillance. Since 1993, there has been an increasing incidence of CJD, from 0.7 in 1993 to 1.6 in 2005 with a quite stable level since 1998. During this period, the proportion of patients with MV and VV codon 129 genotype rose, possibly because of better identification of atypical subtypes. Six percent of all patients had a PRNP mutation, mainly D178N-129M (FFI), E200K and V210I. Iatrogenic CJD was a rare phenomenon. No patient infected by cadaveric growth hormone extracts was reported. Furthermore, no variant CJD patient has yet been identified in Germany. Differential diagnoses revealed a variety of neurodegenerative diseases, with Alzheimer's disease in the lead. One-third of the non-CJD patients included in this study suffered from a potentially treatable disorder such as metabolic or inflammatory diseases. The incidence and mortality rates in Germany are similar to those in other European countries. In contrast, however, acquired forms, such as iatrogenic and variant CJD are still rare in Germany or have not yet been identified.
Key Words: CJD; dementia; epidemiology; diagnosis; CSF; MRI; codon 129 genotype; genetic CJD; reversible/treatable dementia
Abbreviations: BSE, bovine spongiform encephalopathy; CJD, Creutzfeldt–Jakob disease; FFI, fatal familial insomnia; GSS, Gerstmann–Straeussler–Scheinker syndrome
Received August 10, 2006. Revised December 25, 2006. Accepted March 8, 2007.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  L. A. Stewart, L. H.M. Rydzewska, G. F. Keogh, and R. S.G. Knight Systematic review of therapeutic interventions in human prion disease Neurology, April 8, 2008; 70(15): 1272 - 1281. [Abstract] [Full Text] [PDF]
|
|