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 ye...
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Elsevier
2025-03-01
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| Series: | Epilepsy & Behavior Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2589986424000893 |
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| author | Xiaorui Liu Tiejia Jiang Lu Xu Weiran Zhang Feng Gao |
| author_facet | Xiaorui Liu Tiejia Jiang Lu Xu Weiran Zhang Feng Gao |
| author_sort | Xiaorui Liu |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-58ed1fd2d8af4e2e83c08c7f9f4f5b43 |
| institution | DOAJ |
| issn | 2589-9864 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | Elsevier |
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| series | Epilepsy & Behavior Reports |
| spelling | doaj-art-58ed1fd2d8af4e2e83c08c7f9f4f5b432025-08-20T02:47:36ZengElsevierEpilepsy & Behavior Reports2589-98642025-03-012910073210.1016/j.ebr.2024.100732Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleepXiaorui Liu0Tiejia Jiang1Lu Xu2Weiran Zhang3Feng Gao4Department of Neurology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Epilepsy Center, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Children’s Hospital, National Clinical Research Center for Child Health, Zhejiang University School of Medicine, Hangzhou 310052, ChinaDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Epilepsy Center, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Children’s Hospital, National Clinical Research Center for Child Health, Zhejiang University School of Medicine, Hangzhou 310052, ChinaDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Epilepsy Center, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Children’s Hospital, National Clinical Research Center for Child Health, Zhejiang University School of Medicine, Hangzhou 310052, ChinaDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Epilepsy Center, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Children’s Hospital, National Clinical Research Center for Child Health, Zhejiang University School of Medicine, Hangzhou 310052, ChinaDepartment of Neurology, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Epilepsy Center, Children’s Hospital, Zhejiang University School of Medicine, Hangzhou 310052, China; Children’s Hospital, National Clinical Research Center for Child Health, Zhejiang University School of Medicine, Hangzhou 310052, China; Corresponding author at: Children’s Hospital, Zhejiang University School of Medicine, 3333 Binsheng Rd, Hangzhou, Zhejiang, China.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.http://www.sciencedirect.com/science/article/pii/S2589986424000893D/EE-SWASESESRefractoryPropofolMidazolam |
| spellingShingle | Xiaorui Liu Tiejia Jiang Lu Xu Weiran Zhang Feng Gao Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep Epilepsy & Behavior Reports D/EE-SWAS ESES Refractory Propofol Midazolam |
| title | Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep |
| title_full | Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep |
| title_fullStr | Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep |
| title_full_unstemmed | Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep |
| title_short | Efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike-wave activation in sleep |
| title_sort | efficacy of propofol and midazolam combination in managing refractory epileptic encephalopathy with spike wave activation in sleep |
| topic | D/EE-SWAS ESES Refractory Propofol Midazolam |
| url | http://www.sciencedirect.com/science/article/pii/S2589986424000893 |
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