NR AOVJ
AU Lagodana,A.; Puopolo,M.; Almonti,S.; Mellina,V.; Ingrosso,L.; Poleggi,A.; Pocchiari,M.
TI Analysis of the Factors Influencing Survival in Human Transmissible Spongiform Encephalopathies
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - PG-30
PT Konferenz-Poster
AB
The objective of this study was to identify variables that have an influence on survival, i.e. the total duration of clinical illness in different forms of human Transmissible Spongiform Encephalopaties (TSE).
Sporadic definite or probable CJD cases (n= 381), and genetic TSE (gTSE) notified to the Register of CJD at the Istituto Superiore di Sanità, Rome Italy, have been included in the analyses of survival. Survival curves were estimated by univariate analysis, the Kaplan Meier method, and by multivariate analysis, the Cox proportional hazard model.
Univariate survival analyses of the sCJD cases showed that the duration of illness is affected by gender (P = .0022), age at onset (P = .0071), and polymorphism at codon 129 (P = .0090).
The multivariate analysis confirmed that survival is longer in sCJD patients whose age at clinical onset was lower than 65 years, and longer in females respect to males.
In gTSE the analysis was performed for the two most frequent mutations, the E200K and the V210I. Univariate survival analysis showed that the survival is shorter in V210I male patients in a statistically significant way. This effect was in the same direction in E200K patients but it missed statistical significance. The age at onset does not affect survival when cut at 58 years. The effect of the polymorphism at codon 129 did not reach a statistical significance, however, it is noteworthy to say that methionine homozigosity seems to induce a short survival in E200K patients, but a long survival in V210I patients.
In this study we have identified as determinant of survival in sCJD sex, age at onset, and polymorphism at codon 129. In the genetic cases these factors seem to influence in the same way the survival but do not reach a statistical significance, probably because of the small number of cases available.
This study clarify variables that can influnce disease pathogenesis as well constitute a background for the assessment of efficacy of novel treatments.
AD A. Lagodana, M. Puopolo, S. Almonti, V. Mellina, L. Ingrosso, A. Poleggi, M. Pocchiari, Istituto Superiore di Sanitá, Italy
SP englisch
PO Deutschland