Acquired epileptiform opercular syndrome evaluated with real-time transcranial Doppler ultrasound-video-electroencephalogram before and after treatment: a case report

Abstract

Background Acquired epileptiform opercular syndrome (AEOS) with electrical status epilepticus during sleep (ESES) may be recurrent and intractable. The real-time transcranial Doppler ultrasound–sleep-deprived video electroencephalogram (TCD-SDvEEG) can be used to observe the relationships among hemodynamic, electrophysiological, and clinical factors in a patient during therapy. This study reported the case of a healthy 5-year-old boy with AEOS. Case presentation The patient had initial seizures during sleep at the age of 1 year, with the left mouth pouting, left eye blinking and drooling for several seconds, and, sometimes, the left upper-limb flexion and head version to the left, lasting for 1–2 min. The combined antiepileptic drug regimens, including valproate, lamotrigine, and clonazepam, failed in the present case. Therefore, the add-on high-dose methylprednisolone therapy was provided. Also, the serial TCD-SDvEEG was used to monitor the dynamic changes before and after add-on steroid treatment. The results showed less than 15% variation in the range of blood flow fluctuation with spikes during non-rapid eye movement sleep after treatment. This was similar to the outcomes in healthy children and also accorded with the clinical improvements such as seizure control, drooling control, and language ability melioration. However, 95% of spike-wave index (SWI) was still maintained. The improvements in cerebral hemodynamics and clinical manifestations were faster and earlier than the SWI progression. Conclusions The real-time TCD-SDvEEG was highly sensitive in detecting therapeutic changes. The findings might facilitate the understanding of the mechanisms underlying neurovascular coupling in patients with AEOS accompanied by ESES.

A follow-up study in children with status epilepticus during sleep: From clinical spectrum to outcome

“…Consequently, continuous and active treatment of seizures, SES, and neuropsychological impairments in children with ABFEC is highly essential. Moreover, our previously reported findings [20,21] suggested that clinical symptoms of CI in IFE patients might be improved when EEG was transformed from comprehensive at initial examination to focal or multifocal at later investigation. Therefore, seizure control and cognitive improvement mainly coexisted in children with SES.…”

“…The therapeutic outcome measures were based on the EEG outcomes as well as clinical response. The decrement of more than 25% in SWI, or transformation of generalized continuous activity into focal continuous activity, was considered as EEG improvement [20,21]. However, in terms of SES pattern being focal or multifocal pattern [22] at initial examination, the EEG improvement was based on the decrement of more than 25% in SWI.…”