Early cortical and late striatal diffusion restriction on 3T MRI in a long‐lived sporadic creutzfeldt–jakob disease case

Multimodal MRI staging for tracking progression and clinical-imaging correlation in sporadic Creutzfeldt-Jakob disease

“…Thus, a possible explanation for the finding of longitudinal decreasing MD within the basal ganglia in Eisenmenger et al, 2016 may be due to the different timing of involvement of the subcortical compared to the cortical GM in sCJD. This is further supported by a recent longitudinal quantitative MRI analysis of a single sCJD subject with seven MRI scans, including five DTI acquisitions, from 4th to 17th months from disease onset ( Vitali et al, 2019 ). This paper showed a pattern of early cortical and later striatum involvement, suggesting cortical-subcortical spreading of the disease (which we find commonly in our clinical experience).…”

“…This paper showed a pattern of early cortical and later striatum involvement, suggesting cortical-subcortical spreading of the disease (which we find commonly in our clinical experience). A finding of further interest in Vitali et al, 2019 was that the early cortical involvement with restricted diffusion was associated with early cognitive impairtment, whereas the late striatal restricted diffusion was associated with late extrapyramidal motor impairment. In the context of a cortical-subcortical spreading, with involvement of subcortical gray matter typically occurring in later stages of the disease, it is conceivable that whereas within the cortex it is possible to detect the full spectrum along the quasi J-shaped curve of MD changes, within the basal ganglia and particularly in the striatum, which are on the earlier parts of the quasi J-shaped curve with reduced MD, one might not be able to detect pseudo-normalization or increased MD at the time of the subjects last MRI or prior to their death.…”

“…This temporal course might prevent use of GM MD alone as an imaging biomarker in treatment trials. Furthermore, GM MD varies even between brain areas in single subjects, likely due to regions being in different disease stages with varying underlying neuropathological or neurophysiological abnormalities ( Grau-Rivera et al, 2017 , Vitali et al, 2019 ).…”