NR AXMY
AU Gelpi,E.; Höftberger,R.; Unterberger,U.; Ströbel,T.; Voigtlaender,T.; Drobna,E.; Jarius,C.; Heinzl,H.; Bernheimer,H.; Budka,H.
TI Creutzfeldt-Jakob Disease in Austria: An Autopsy-controlled Study
QU International Conference - Prion 2007 (26.-28.9.2007) Edinburgh International Conference Centre, Edinburgh, Scotland, UK - Book of Abstracts: Epidemiology, Risk Assessment and Transmission P04.60
IA http://www.prion2007.com/pdf/Prion Book of Abstracts.pdf
PT Konferenz-Poster
AB
Background: Definite diagnosis of transmissible spongiform encephalopathies (TSEs) requires neuropathology, usually at autopsy. Epidemiology of human TSEs has relied on definite as well as "probable" cases in which neuropathological confirmation is lacking, usually because of low autopsy rates in most countries.
Aim: To report the most relevant epidemiological data of autopsy confirmed human TSEs in Austria during a 10-year surveillance period (1996-2006). Since postmortem investigation of brain tissue is mandatory in every suspect case of TSE, our data may serve as autopsy-controlled baseline for comparison with countries with lower autopsy rate.
Methods: Data have been prospectively collected at the Austrian Reference Centre for Human Prion Diseases [ÖRPE] since 1996. Classification of referrals has been done according to WHO surveillance criteria. Laboratory tests include 14-3-3-analysis in CSF, EEG, postmortem investigation of brain tissue, and sequencing of the prion protein gene to identify mutations and to define the genotype at codon 129.
Results: Over 900 referrals have been registered by ÖRPE during this period. The total number of TSE cases since 1969 is 206. The average yearly mortality from 1996 to 2006 is 1,39 per million, with a peak incidence of 2,7 in 2006. 85% of definite TSEs were classified as sporadic Creutzfeldt-Jakob disease (sCJD). We observed a significant linear increase of the mean age at death of 0,6 years per calendar year (p = 0,016). Based on autopsy results, clinical diagnostic surveillance criteria had sensitivity and specificity, respectively, of 82,7% and 80,0% for "probable" CJD, and a positive predictive value of 80,5% for "probable" and of 38,9% for "possible" CJD. Alternative neuropathological diagnoses included Alzheimer's disease with or without Lewy body pathology, vascular encephalopathy, metabolic encephalopathies, and viral or limbic encephalitis. No common risk factors have been detected. However, CJD mortality is in Vienna double as high as in the rest of the country.
Conclusion: The steady increase of mortality rates especially in old age groups reflects most likely improved case ascertainment due to active surveillance causing higher awareness of the medical community. In comparison with other European countries, it is re-assuring to note that the overall death rate of TSEs does not significantly differ from the Austrian autopsy-controlled data, thus confirming the value of clinical surveillance criteria.
AD E. Gelpi, R. Hoeftberger, U. Unterberger, T. Stroebel, T. Voigtlaender, E. Drobna, C. Jarius, H. Bernheimer, H. Budka, Institute of Neurology, Medical University of Vienna, Austria; H. Heinzl, Medical University Vienna, Core Unit for Medical Statistics and Informatics, Austria
SP englisch
PO Schottland