Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave
Xiaorui Liu1,2,3, Tiejia Jiang1,2,3, Lu Xu1,2,3
1Department of Neurology, Children's Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China.
Insights
Refractory Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep (ESES) in a child was effectively treated with propofol and midazolam. This combination therapy led to significant improvement in EEG findings and cognitive function over a three-year follow-up.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Neurophysiology
Background:
- Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep (ESES) is a severe condition characterized by frequent seizures and cognitive decline.
- Management of refractory ESES remains challenging, with limited treatment options and potential for irreversible neurological deficits.
- The case highlights the progressive nature of ESES and its impact on a young patient's development.
Abstract:
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.
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