Fig. 1

MRI findings. a, b MRI findings in patient 1 at the onset of acute encephalopathy (AE). Reduced water diffusion was evident over the entire cerebral cortex and the posterior parts of the thalami (a diffusion-weighted images; b apparent diffusion coefficient map). c–e MRI of patient 1 performed 4 days after AE onset. Marked brain edema with narrowing of the lateral ventricles was seen on T1- (c) and T2-weighted (d) images. Diffusion-weighted images exhibited reduced water diffusion, predominantly in the subcortical white matter (e). f MRI of patient 3 performed 7 days after AE onset. Diffusion-weighted images revealed reduced water diffusion in the subcortical white matter and corpus callosum. g MRI of patient 4 performed 3 days after AE onset. Reduced water diffusion was evident in the subcortical white matter of the left frontal and bilateral temporo–parieto–occipital areas. h MRI of patient 6 performed 5 days after AE onset. Reduced water diffusion was evident in the bilateral thalami and subcortical white matter, except for the bilateral occipital areas. i MRI of patient 2 performed 17 days after AE onset. Diffusion-weighted images revealed reduced water diffusion in the caudate nuclei, basal ganglia, and the pulvinar thalami