NR APAL
AU Westner,I.; Neukirch,K.; Windl,O.; Grasbon-Frodl,E.; Krebs,B.; Poser,S.; Zerr,I.; Kretzschmar,H.A.
TI 10 Years of German CJD Surveillance: A neuropathological and epidemiological view
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-72
PT Konferenz-Poster
AB
Funded by the Federal Ministry of Health (Bundesgesundheitsminsterium), the German CJD surveil-lance group started its work in June 1993. To date more than 2100 CJD suspect cases have been examined clinically and/or neuropathologically following a standardized protocol according to the WHO criteria for prion diseases.
We have created a network that includes hospitals, general practitioners and pathologists. The Institute of Neuropathology in Munich cooperates with the clinical part of the study in Göttingen and co-ordinates the autopsies of deceased CJD suspect cases in Germany and performs the neuropathologic, genetic and Western blot diagnosis.
In the last 10 years we have diagnosed 595 sporadic CJD (sCJD) cases, 6 iatrogenic cases (iCJD) and familiar cases including 17 patients with Gerstmann-Sträussler-Scheinker Syndrome (GSS), 28 cases with Fatal Familiar Insomnia (FFI) and over 40 familial CJD cases. A number of novel mutations of the prion protein gene (PRNP) associated with familial CJD have been identified. The paraffin embedded tissue blot (PET blot) has been established as a valuable tool in the neuropathologic diagnosis of human prion diseases.
The mortality of sCJD in Germany has been in the range of 0.7 to 1.3 per year per million population (including definite and probable cases). Cases of variant CJD (vCJD) have not been identified in Germany.
The brain bank contains more than 1000 formalin fixed brains and over 450 frozen specimens of hu-man CJD cases. Tissue samples are provided to research groups following the regulations of the BrainNet (the brain bank consortium in Germany) and the BrainNet Europe.
AD Ingo Westner, Karin Neukirch, Eva Grasbon-Frodl, Bjarne Krebs, Hans Kretzschmar, LMU München, Institute of Neuropathology, Germany; Otto Windl, VLA Weybridge, UK; Sigrid Poser, Inga Zerr, University of Göttingen, Dep. of Neurology, Germany
SP englisch
PO Deutschland