NR AUSO
AU Heinemann,U.; Krasnianski,A.; Meissner,B.; Varges,D.; Bartl,M.; Stoeck,K.; Kallenberg,K.; Schulz-Schaeffer,W.J.; Steinhoff,B.J.; Kretzschmar,H.A.; Zerr,I.
TI Creutzfeldt-Jakob disease in Germany: a prospective 12-years surveillance
QU TSE-Forum, 6. Kongress - Nationale TSE-Forschungsplattform, Greifswald 26.6.-28.6.2006, Poster: Diagnostik DIA-04
PT Konferenz-Poster
AB
Background and Methods:
Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disorder with a worldwide incidence of 1-1.5 per million. As in other countries, in 1993 a CJD Surveillance Unit in Göttingen (Germany) with clinical and neuropathological approach was established. Here we report the epidemiological data of a prospective 12-year-surveillance.
Results:
Since 1993, there is an increasing incidence from 0.7 in 1993 to 1.7 in 2004. During this period, also the proportion of the MV and VV subtype was rising as a possible explanation with better identification of atypical subtypes. Additionally, 9% genetic CJD (gCJD) was noticed with mainly D178N (resulting in FFI), E200K and V210I mutations. Iatrogenic CJD was a rare phenomenon. No patient infected by cadaveric growth hormones was described. Further, no variant CJD patient is yet identified in Germany. Differential diagnoses revealed a variety of neurodegenerative diseases led by Alzheimer's disease. One third of the non-CJD patients included in this study suffered from a potentially treatable disorder such as metabolic or inflammatory diseases.
Conclusion:
The epidemiological Surveillance in Germany revealed similar incidences as in other European countries. In contrast to other countries, infectious forms as iatrogenic CJD and variant CJD are still rare or not yet identified.
AD Uta Heinemann, Anna Krasnianski, Bettina Meissner, Daniela Varges, Mario Bartl, Katharina Stoeck, Inga Zerr, National Reference Center for transmissible spongiform encephalopathies, Department of Neurology, Georg-August University Göttingen, Germany; Kai Kallenberg, Department of Neuroradiology, Georg-August University Göttingen, Germany; W.J.Schulz-Schaeffer, Department of Neuropathology, Georg-August University Göttingen, Germany; B.J. Steinhoff, Epilepsy Center Kork, Diakonie Kork, Germany; Hans Kretzschmar, Department of Neuropathology, Ludwig-Maximilian University Munich, Germany
SP englisch
PO Deutschland
OR Tagungsband