PMC8219739_01
A timeline presenting the medical history including seizure burden, neuroimaging findings and most important treatment.
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A timeline is shown in the Fig. 1.
Axial T2 and coronal FLAIR MR images 2008 (age 32 years)(a and b), 2013 (age 37 years)(c and d), June 2019 (e and f) and September 2019 (age 43 years)(g and h). First examination (a and b) showed focal frontal right-sided cortical edema (arrows), otherwise normal. Second examination in 2013 (c and d) revealed right sided hemiatrophy and status after subpial resection. In June 2019 (e and f) there was progressive hemiatrophy and extensive parietooccipital cortical edema (arrows). The edema had subsided in September 2019 (g and h).
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An MRI scan of the brain showed seizure-related signal changes consistent with edema in the right parietotemporooccipital region and posteriomedially in the right thalamus in addition to progressive hemiatrophy (Fig. 2).
A) Longitudal EEG recording demonstrating marked epileptic activity during a seizure shortly before treatment with anakinra. The trend analysis (spectrogram) shown on top shows four epileptic seizures within the 30 min registration period. B) Longitudinal EEG recording 6 weeks after start of anakinra with no ongoing epileptic activity. No seizures are now seen in the 30 min trend analysis.
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EEG now showed occasional independent seizures arising from the left frontal region, in addition to focal status epilepticus in the right temporoparietal region (Fig. 3a). Within 1-2 days after injection, seizure frequency decreased significantly, and, after one week, the seizures stopped completely followed by a marked improvement in EEG (Fig. 3b).