Uchida Takashi, Takayanagi Masaru, Kitamura Taro, Nishio Toshiyuki, Numata Yurika, Endo Wakaba, Haginoya Kazuhiro, Ohura Toshihiro
Division of Pediatrics, Sendai City Hospital, Sendai, Japan.
Department of Pediatrics, Ishinomaki Red Cross Hospital, Ishinomaki, Japan.
Pediatr Int. 2016 Aug;58(8):750-3. doi: 10.1111/ped.12934. Epub 2016 Jun 8.
Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is characterized by repetitive seizures during the acute and chronic phases and has a poor neurological outcome. Burst-suppression coma via continuous i.v. infusion of a short-acting barbiturate is used to terminate refractory seizures, but the severe side-effects of short-acting barbiturates are problematic. We report on a 9-year-old boy with AERRPS who was effectively treated with very-high-dose phenobarbital (VHDPB) combined with intermittent short-acting barbiturates. VHDPB side-effects were mild, especially compared with those associated with continuous i.v. infusion of short-acting barbiturates (dosage, 40-75 mg/kg/day; maximum blood level, 290 μg/mL). Using VHDPB as the main treatment, short-acting barbiturates were used intermittently and in small amounts. This is the first report to show that VHDPB, combined with intermittent short-acting barbiturates, can effectively treat AERRPS. After treatment, convulsions were suppressed and daily life continued, but intellectual impairment and high-level dysfunction remained.
伴有难治性反复部分性癫痫发作的急性脑炎（AERRPS）的特征是在急性期和慢性期出现反复癫痫发作，且神经学预后较差。通过持续静脉输注短效巴比妥酸盐诱导爆发抑制昏迷用于终止难治性癫痫发作，但短效巴比妥酸盐的严重副作用存在问题。我们报告了一名9岁患有AERRPS的男孩，他接受了大剂量苯巴比妥（VHDPB）联合间歇性短效巴比妥酸盐治疗，效果良好。VHDPB的副作用较轻，尤其是与持续静脉输注短效巴比妥酸盐（剂量为40 - 75mg/kg/天；最高血药浓度为290μg/mL）相比。以VHDPB作为主要治疗方法，间歇性少量使用短效巴比妥酸盐。这是首次表明VHDPB联合间歇性短效巴比妥酸盐可有效治疗AERRPS的报告。治疗后，惊厥得到抑制，日常生活得以继续，但仍存在智力障碍和高级功能障碍。
