PMC7745547_01
Initial EEG revealed delta-beta complexes (A). Electrographic seizures onset predominantly in the right temporal (B) and left temporal (C) regions.
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Interictally, intermittent delta waves with overriding 15-16 Hz beta activity were observed, resembling interictal discharges described in other children with FIRES (Figure 1A). Initial CSF analysis was normal.
Serial MRIs on day 7 (A-C) and 16 (D-F) showed progressive T2 (C, F)/FLAIR (A, D) hyperintensities with diffusion restriction (B, E) in the right hippocampus and generalized atrophy.
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Serial MRIs (Figure 2) showed progressive T2/FLAIR hyperintensities with diffusion restriction in the right hippocampus.
Seizure burden displayed as approximate seizures/day and daily seizure time (minutes/day) is summarized. Notably, seizure burden on days 19-22 was nearly continuous at times and challenging to quantify, and thus represented as 500 seizures and seizure minutes/day (A). Administration of immunotherapy, ketogenic diet, (A) continuous infusions, and maintenance anti-seizure medications is highlighted (B). There was a decrease in approximate seizures/day and daily seizure time (minutes/day) within 3 days of tocilizumab and 9 days of KD initiation (A). Decreased seizure burden allowed for weaning of continuous infusions (B).
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Seizures became near continuous prompting 2 trials of burst suppression and remained refractory to midazolam (2.9 mg/kg/hour), ketamine (100 mcg/kg/minute or 6 mg/kg/hour), pentobarbital (6 mg/kg/hour), IV methylprednisolone (30 mg/kg daily for 5 days), IVIg (2 g/kg over 2 days), and anakinra (titrated to 20 mg/kg/day, hospital day 6-20) (Figure 3). Maintenance ASMs were sequentially added, including levetiracetam (100 mg/kg/day), phenobarbital (13 mg/kg/day), valproic acid (40 mg/kg/day), lacosamide (20 mg/kg/day), topiramate (10 mg/kg/day), cannabidiol (20 mg/kg/day), and lorazepam (1.5 mg/kg/day) (Figure 3B). KD was initiated on day 14, and a beta-hydroxybutyric acid level of >1.5 mmol/L was obtained on day 16 and sustained until discontinuation on day 63 (Figure 3A).