Abstracts

Rationale: To explore treatment strategies for refractory Epileptic Encephalopathy with Spike-Wave Activation in Sleep (EE-SWAS) and propose potential therapeutic options.


Methods: We presented a case detailing the characteristics and treatment outcomes of a patient with refractory EE-SWAS who underwent combined therapy with propofol and midazolam at the Department of Pediatric Neurology, Zhejiang University School of Medicine Children’s Hospital, in November 2020.


Results: The patient, a 7-year-old boy with a history of cerebral hemorrhage occurring 10 days after birth primarily involving the right thalamus, began experiencing seizures at the age of 2. He was initially treated with multiple antiseizure medications (ASMs), which controlled the seizures for almost three years. In June 2019, at the age of 5, seizures recurred, and his electroencephalography (EEG) began to show signs of electrical status epilepticus in sleep (ESES), with a spike-wave index (SWI) of approximately 85% during non-rapid eye movement (NREM) sleep. Following initial high-dose methylprednisolone pulse therapy, the discharge index decreased to 50% but relapsed shortly thereafter. A follow-up EEG after more than six months revealed that the SWI had risen again to 80%. Despite subsequent treatments with high-dose methylprednisolone, midazolam infusion, and four combined ASMs, the SWI remained between 75% and 85%. His cognitive impairment progressively worsened following discharge, and he progressed to a somnolent state six months later, becoming unresponsive to verbal stimuli and unable to engage in simple communication, with a SWI of more than 85%, nearly continuous discharges. During hospitalization, the patient received a combination of propofol and midazolam infusion in the intensive care unit (ICU), resulting in significant improvement. The SWI decreased to 50% upon completion of the treatment period, and no seizures occurred. Over a follow-up period of more than three years, EEG still showed no signs of ESES, and no seizures were reported. The patient's cognitive function showed notable improvement.


Conclusions: 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 remarkably effective suppression of ESES phenomena, presenting a promising treatment strategy for refractory ESES.


Funding: N/A