Prognostic value of diffusion-weighted imaging in patients with newly diagnosed sporadic Creutzfeldt-Jakob disease

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 (kappa = 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.

Automated summary

The study identified age and diffusion restriction in the caudate nucleus and putamen as independent prognostic factors for poor overall survival in patients with sporadic Creutzfeldt-Jakob disease. The DWI prognostic score model based on these features may be useful for disease stratification in clinical settings, with excellent interobserver agreement.