NR ATKQ
AU Puopolo,M.; Ladogana,A.; Pocchiari,M.
TI A model to predict PrPtse type in living patients
QU International Conference - Prion 2005: Between fundamentals and society's needs - 19.10.-21.10.2005, Congress Center Düsseldorf - Poster Session: Diagnosis DIA-28
PT Konferenz-Poster
AB
PrPtse type strongly influences survival of sporadic Creutzfeldt-Jakob disease (CJD) patients [Pocchiari et al., 2004]. However, PrPtse type is usually only available post mortem. A multivariate logistic regression was carried out aiming at defining a probabilistic criteria to classify sporadic CJD as PrPtse type 2a on the basis of EEG, 14-3-3 protein and codon 129 polymorphism. Data on sCJD patients were taken from the EUROCJD database.
Four hundred and twenty seven sCJD cases were included in the analyses. Univariate analyses showed that PrPtse type 2a cases presented an atypical EEG pattern (79%), a positive protein 14-3-3 in the CSF (84%) and valine homozygosity (53%) for polymorphism at codon 129 of the PRNP gene. PrPtse type1 cases showed a typical EEG pattern (73%), a positive protein 14-3-3 in the CSF (95%) and methionine homozygosity (89%). The multivariate logistic regression analysis showed that the three variables contributed independently and significantly to correctly classify PrPtse type 2a patients (and for exclusion PrPtse type 1 patients). A predictive model with a sensibility of 0.80 (and specificity = 0.94, Positive Predictive Value = 0.84, Negative Predictive Value = 0.92) classifies PrPtse type2a patients:
- all VV cases, independently from EEG pattern and/or presence of protein 14-3-3 in the CSF;
- MV cases with atypical EEG, independently from the presence of the protein 14-3-3 in the CSF;
- MV cases with typical EEG, and negativity to the protein 14-3-3 in the CSF.
This model correctly classifies 90% of all cases and it might be improved by including other clinical or laboratory parameters (such as MRI, tau, etc.).
Acknowledgements
We wish to thank all participants to the Eurocjd group for collecting data.
Reference
Pocchiari M, et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain, 127: 2348-59, 2004.
AD M.Puopolo, A.Ladogana, M.Pocchiari, Istituto Superiore di Sanità, Italy
SP englisch
PO Deutschland