Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep

We presented a 7-year-old boy with refractory Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep (EE-SWAS) successfully managed with a combination of propofol and midazolam. His seizures began at age 2, initially controlled by multiple antiseizure medications (ASMs) for almost three years. At age 5, seizures recurred with electroencephalography (EEG) showing electrical status epilepticus in sleep (ESES) and a spike-wave index (SWI) of 85 %. High-dose methylprednisolone pulse therapy initially reduced the SWI to 50 %, but it relapsed to 80 % within six months. Despite further treatments, including methylprednisolone, midazolam infusion, and four combined ASMs, the SWI persisted between 75 % and 85 %, leading to progressively worsening cognitive impairment and subsequently a somnolent state with nearly continuous discharges. During hospitalization, a combination of propofol and midazolam significantly improved the condition, reducing the SWI to 50 % upon completion of the treatment period. Over a three-year follow-up, no ESES or seizures were reported, and cognitive function notably improved. Currently, there is no consensus on the treatment of ESES, which is sometimes refractory to medication and can result in partially irreversible cognitive impairment. Propofol in combination with midazolam has demonstrated effective suppression of ESES phenomena, presenting a promising treatment strategy for refractory ESES.