Multicentre multiobserver study of diffusion-weighted and fluid-attenuated inversion recovery MRI for the diagnosis of sporadic Creutzfeldt-Jakob disease: a reliability and agreement study

Abstract

Objectives To assess the utility of the display standardisation of diffusion-weighted MRI (DWI) and to compare the effectiveness of DWI and fluid-attenuated inversion recovery (FLAIR) MRI for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD). Design A reliability and agreement study. Setting Thirteen MRI observers comprising eight neurologists and five radiologists at two universities in Japan. Participants Data of 1.5-Tesla DWI and FLAIR were obtained from 29 patients with sCJD and 13 controls. Outcome measures Standardisation of DWI display was performed utilising b0 imaging. The observers participated in standardised DWI, variable DWI (the display adjustment was observer dependent) and FLAIR sessions. The observers independently assessed each MRI for CJD-related lesions, that is, hyperintensity in the cerebral cortex or striatum, using a continuous rating scale. Performance was evaluated by the area under the receiver operating characteristics curve (AUC). Results The mean AUC values were 0.84 (95% CI 0.81 to 0.87) for standardised DWI, 0.85 (95% CI 0.82 to 0.88) for variable DWI and 0.68 (95% CI 0.63 to 0.72) for FLAIR, demonstrating the superiority of DWI (p<0.05). There was a trend for higher intraclass correlations of standardised DWI (0.74, 95% CI 0.66 to 0.83) and variable DWI (0.72, 95% CI 0.62 to 0.81) than that of FLAIR (0.63, 95% CI 0.53 to 0.74), although the differences were not statistically significant. Conclusions Standardised DWI is as reliable as variable DWI, and the two DWI displays are superior to FLAIR for the diagnosis of sCJD. The authors propose that hyperintensity in the cerebral cortex or striatum on 1.5-Tesla DWI but not FLAIR can be a reliable diagnostic marker for sCJD.

Figure 1

Receiver operating characteristic curves for each display in diagnosis of sporadic Creutzfeldt–Jakob disease. (A) Neurologists, (B) radiologists and (C) all observers. The true rate (sensitivity) is plotted as a function of the false-positive rate (1 − specificity). DWI, diffusion-weighted imaging; FLAIR, fluid-attenuated inversion recovery; sDWI, standardised DWI; vDWI, variable DWI.

Figure 2

Representative MRI of a sporadic Creutzfeldt–Jakob disease patient (case 17). Abnormal hyperintensity in the cerebral cortex is evident on standardised diffusion-weighted imaging (A, arrow) but obscure on fluid-attenuated inversion recovery (B, arrow).

Figure 3

Intraclass correlations for each display. Error bars represent upper limits of 95% CIs. DWI, diffusion-weighted imaging; FLAIR, fluid-attenuated inversion recovery; sDWI, standardised DWI; vDWI, variable DWI.