PMC7303322_01
(A) Polygraphic video-EEG recording of Case #1, showing repetitive and continuous myoclonic jerks with an EEG counterpart characterized by diffuse repetitive spikes and multiple artifacts related to continuous myoclonic jerks of the face muscles. (B) Polygraphic recording after VNS implantation and after remission of myoclonic SE, showing low-voltage activity and rare myoclonic jerks on the EMG channels. (C) Coronal T2 and axial FLAIR MRI of Case #1 during RSE, showing mild cerebral atrophy, cerebellar atrophy, and hyperintensity over the right frontal region. (D) EEG during SE showing continuous epileptiform abnormalities over the left hemisphere and multiple repetitive spikes of Case #2. (E) EEG from 15 days after VNS implantation: epileptiform abnormalities were significantly reduced; some spikes over left temporal regions are evident. (F) Axial and coronal T2 brain MR showing cerebral atrophy and a simplification of cortical gyri, more evident over bilateral frontal and central regions of Case #2.
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F1
EEG recordings were consistently characterized by continuous epileptiform discharges over the right fronto-central area with bilateral diffusion (Figure 1A). MR showed a hyperintensity on fluid-attenuated inversion recovery (FLAIR) sequences involving the right frontal region (Figure 1C). Metabolic workup was negative. SRSE remitted after 7 days and did not recur (Figure 1B). Brain MR showed bilateral frontal simplification of cortical gyri at the age of 2 months, together with progressive diffuse cerebral atrophy at the age of 8 months (Figure 1F). SRSE onset, VNS (Demipulse 103 Cyberonics) was implanted. SE remitted after 10 days and did not recur (Figures 1D,E).