Fukuyama Tetsuhiro, Inaba Yuji, Higuchi Tsukasa, Sekiguchi Yukio, Ishida Shuichi
Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Nagano.
No To Hattatsu. 2011 Jul;43(4):313-6.
Acute encephalitis with refractory, repetitive partial seizures (AERRPS) is a peculiar form of encephalitis mainly affecting children. Although not usually lethal, we report a case of sudden unexpected death in epilepsy (SUDEP) 3 years after the onset of AERRPS. A 6-year-old boy was admitted to our hospital because of fever and extremely refractory partial and secondary generalized seizures with delirium and psychiatric change. The seizures were highly resistant to anticonvulsants and suppressed only by large dose intravenous administration of midazolam. Seven months after the onset, the seizures were ameliorated by treatment with potassium bromide and clorazepate. After the acute phase, the patient developed complex partial seizures that tended to present with cyanosis. At the age of 10, he was found lying prone in respiratory arrest with facial pallor. Although he regained cardiac function after being taken to our emergency room, the patient died 12 days later. Six SUDEP cases after the onset of AERRPS, including this one, have been reported to date. Since epilepsy following AERRPS is one of the risk factors of SUDEP, clinicians should consider SUDEP to be a rare but high risk syndrome in AERRPS-afflicted children.
伴有难治性、反复性部分性发作的急性脑炎（AERRPS）是一种主要影响儿童的特殊脑炎形式。虽然通常不会致命，但我们报告了1例AERRPS发病3年后癫痫性猝死（SUDEP）的病例。一名6岁男孩因发热、伴有谵妄和精神改变的极其难治性部分性发作及继发性全身性发作而入住我院。这些发作对抗惊厥药高度耐药，仅通过大剂量静脉注射咪达唑仑才能得到抑制。发病7个月后，通过溴化钾和氯氮卓酯治疗，发作得到改善。急性期过后，患者出现复杂部分性发作，常伴有发绀。10岁时，他被发现俯卧，呼吸骤停，面色苍白。尽管被送往我们的急诊室后恢复了心功能，但患者12天后死亡。包括本例在内，迄今已报告了6例AERRPS发病后的SUDEP病例。由于AERRPS后的癫痫是SUDEP的危险因素之一，临床医生应将SUDEP视为AERRPS患儿中一种罕见但高风险的综合征。
