LAUSR

Objectives To evaluate clinico-radiologic markers that predict poor overall survival (OS) in sporadic Creutzfeldt-Jakob disease (sCJD) and to develop a prognostic model. Materials and methods Patients with newly diagnosed sCJD were included who underwent diffusion-weighted imaging (DWI) from February 2000 to July 2020. The impact of 9 clinico-radiologic features on OS was analyzed using univariable and multivariable Cox proportional hazards regression model. The DWI prognostic score model was generated. The weighted kappa was calculated for interobserver agreement. Results Sixty patients (mean age ± SD, 61.0 ± 9.7 years, 32 women) were included. Univariable analysis showed positive associations between poor OS and patient age ( p  = 0.003), extent of involved cortical lobes ( p  = 0.11), involvement of caudate nucleus ( p  = 0.07), and putamen ( p  = 0.04). Multivariable analysis demonstrated two independent prognostic factors: age ≥ 60 (HR 2.65, 95% CI, 1.41–4.98), and diffusion restriction in caudate nucleus and putamen (HR 2.24, 95% CI, 1.15–4.37). Based on these features, the DWI prognostic score model was generated: low-risk (0–1 point), intermediate-risk (2–3 points), and high-risk (4–5 points) groups. Median OS in high-risk group was 1.7 months, which was significantly shorter than those in the intermediate-risk (14.2 months) and low-risk (26.5 months) groups ( p  < 0.001). Interobserver agreements were excellent ( κ  = 0.91–0.92). Conclusions Our study demonstrated that age and diffusion restriction in caudate nucleus and putamen were the independent prognostic factors of poor overall survival in sporadic Creutzfeldt-Jakob disease. Our DWI prognostic score model may be useful in clinical settings for disease stratification. Key Points • Age ≥ 60, and diffusion restriction in caudate nucleus and putamen were the independent prognostic factors of poor overall survival in sCJD . • Based on our DWI prognostic score model, median overall survival in high - risk group was 1.7 months, which was significantly shorter than those in the intermediate - risk group (14.2 months) and low - risk group (26.5 months) (p < 0.001). • The proposed DWI prognostic score model may be useful in clinical settings for disease stratification .