PMC8383053_01
The composite figure illustrates MRI T2 (left) and DWI (right) sequences (A) 2 months prior to the presentation of IHHE, multifocal tubers with foci of T2 prolongation in the bilateral cerebral hemispheres, some of which showed linear morphology toward the ventricles; lining the lateral ventricles there are ependymal/subependymal nodules; (B) hospital day 2, mild right hemispheric edema; (C) hospital day 10, diffuse swelling and restricted diffusion of the entire right hemisphere; (D) 1 year after the presentation of IHHE, diffuse right hemispheric encephalomalacia.
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MRI showed subependymal nodules and subcortical tubers (Fig. 1A). Brain MRI showed mild right hemispheric edema (Fig. 1B). IV boluses of lorazepam, levetiracetam, and valproic acid. In this setting, brain MRI was repeated on hospital day 10 and showed diffuse swelling and restricted diffusion of the entire right hemisphere (Fig. 1C). Brain MRI one year after presentation showed diffuse encephalomalacia of the right hemisphere with associated ex vacuo dilatation of the lateral ventricle (Fig. 1D).
EEG (anterior-posterior bipolar montage) reveals evolution from right lateralized periodic discharges maximal in the fronto-temporal region (A) to an electrographic seizure invovling the right hemisphere (B).
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EEG showed right hemispheric seizures with onset in the right fronto-temporal region and spread to the entire right hemisphere (Fig. 2).