Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies

Abstract

A collaborative study of human transmissible spongiform encephalopathies has been carried out from 1993 to 2000 and includes data from 10 national registries, the majority in Western Europe. In this study, we present analyses of predictors of survival in sporadic (n = 2304), iatrogenic (n = 106) and variant Creutzfeldt-Jakob disease (n = 86) and in cases associated with mutations of the prion protein gene (n=278), including Gerstmann- Sträussler-Scheinker syndrome (n = 24) and fatal familial insomnia (n = 41). Overall survival for each disease type was assessed by the .Kaplan-Meier method and the multivariate analyses by the Cox proportional hazards model. In sporadic disease, longer surviva..