PMC6349012_01
Neuroimaging and EEG features of index case.. Legend: Panel A depicts cortical signal increases (white arrows) at baseline, with a right temporal porencephalic cyst, Panel B shows EEG samples, including an "interictal sample" with clonic muscle artifact (thick black arrow), and example of ictal discharge lateralized to the right hemisphere, and Panel C demonstrates subtraction ictal SPECT scans indicating increased perfusion over the course of her focal status epilepticus (performed 4 weeks apart).
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Her interictal EEG demonstrated periodic epileptic discharges at 0.5-2 Hz frequency over the right posterior temporal and centroparietal regions (Fig. 1B, upper panel), which were rarely time-locked with the facial clonic artifact. An ictal SPECT was performed, which revealed right centroparietal and bilateral premotor activations (Fig. 1C, upper panel). In the meantime, on scalp EEG, as well as repeat MRI and ictal SPECT scans of the brain, there was evidence of increased involvement of the insula and frontal lobes, including the contralateral hemisphere, but still no evidence of atrophy on MRI (Fig. 1, lower panels).
PMC6349012_02
Neuroimaging and EEG features of index case.. Legend: Panel A depicts cortical signal increases (white arrows) at baseline, with a right temporal porencephalic cyst, Panel B shows EEG samples, including an "interictal sample" with clonic muscle artifact (thick black arrow), and example of ictal discharge lateralized to the right hemisphere, and Panel C demonstrates subtraction ictal SPECT scans indicating increased perfusion over the course of her focal status epilepticus (performed 4 weeks apart).
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PMC6349012_02_gr1.jpg
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Her interictal EEG demonstrated periodic epileptic discharges at 0.5-2 Hz frequency over the right posterior temporal and centroparietal regions (Fig. 1B, upper panel), which were rarely time-locked with the facial clonic artifact. An ictal SPECT was performed, which revealed right centroparietal and bilateral premotor activations (Fig. 1C, upper panel). In the meantime, on scalp EEG, as well as repeat MRI and ictal SPECT scans of the brain, there was evidence of increased involvement of the insula and frontal lobes, including the contralateral hemisphere, but still no evidence of atrophy on MRI (Fig. 1, lower panels).
Histopathological findings on frontal lobe biopsy.. Legend: Panel A illustrates lymphocytes (CD68, 20 x and 40 x, respectively); Panels B, C, and D illustrate microglia activation (HE) as do Panels C and D (CD68, 20 x and 49 x, respectively).
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RS was confirmed histopathologically in frontal lobe specimens acquired by an open biopsy anterior to the laser ablation tracts (Fig. 2).