A prognostic model for overall survival in sporadic Creutzfeldt-Jakob disease

Introduction: We developed a prognostic model for overall survival after diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) using data from a German surveillance study. Methods: We included 1226 sCJD cases (median age 66 years, range 19-89 years; 56.8% women with information on age, sex, codon 129 genotype, 14-3-3 in the cerebrospinal fluid (CSF), and CSF tau concentrations. The prognostic accuracy for overall survival was measured by the c statistics of multivariable Cox proportional hazard models. A score chart was derived to predict 6-month survival and median survival time. Results: A model containing age, sex, codon 129 genotype, and CSF tau (with two-way interactions) was selected as the model with the highest c statistic (0.686, 95% confidence interval: 0.665-0.707) in a cross-validation approach. Discussion: We developed the first prognostic model for overall survival of sCJD patients based on readily available information only. The developed score chart serves as a hands-on prediction tool for clinical practice.