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. 2020 Oct;16(10):1438-1447.
doi: 10.1002/alz.12133. Epub 2020 Jul 2.

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

Franc Llorens  1   2   3 , Nicole Rübsamen  4 , Peter Hermann  3 , Matthias Schmitz  3 , Anna Villar-Piqué  1   2   3 , Stefan Goebel  3 , André Karch  4 , Inga Zerr  3   5
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A prognostic model for overall survival in sporadic Creutzfeldt-Jakob disease

Franc Llorens et al. Alzheimers Dement. 2020 Oct.

Abstract

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.

Keywords: biomarker; cerebrospinal fluid; neurodegeneration; prognosis; sporadic Creutzfeldt-Jakob disease; tau.

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References

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