第19巻 2023年4月　第2号

■症例報告

カルバマゼピン投与によるミオクロニー発作増悪時にDoose症候群と診断しエトスクシミドが著効した3歳男児
大��　永字¹, 宮本　雄策³, 橋本　康司¹, 原田　こと葉¹, 田嶋　華子¹, 小川　樹里¹, 早川　潤¹, 川上　康彦², 右田　真^1
1日本医科大学武蔵小杉病院小児科
²日本医科大学多摩永山病院小児科
³聖マリアンナ医科大学小児科

A Case of Doose Syndrome Successfully Treated with Ethosuximide in a 3-Year-Old Boy Diagnosed after Carbamazepine Administration Induced Myoclonic Seizures
Eiji Otaka¹, Yusaku Miyamoto³, Koji Hashimoto¹, Kotoha Harada¹, Hanako Tajima¹, Juri Ogawa¹, Jun Hayakawa¹, Yasuhiko Kawakami² and Makoto Migita^1
1)Department of Pediatrics, Nippon Medical School, Musashi Kosugi Hospital
²⁾Department of Pediatrics, Nippon Medical School, Tama Nagayama Hospital
³⁾Department of Pediatrics, St. Marianna University School of Medicine

A 3-year-old boy with a history of four febrile convulsions since the age of 1 year was admitted to the hospital after experiencing a cluster of tonic seizures. Treatment was started with valproic acid (VPA), which proved to be ineffective, so carbamazepine (CBZ) was administered instead. This was judged to be effective because the tonic seizures disappeared temporarily. The patient was discharged, but he then had a similar cluster of tonic seizures and was consequently readmitted. Zonisamide (ZNS) was administered, which resolved the tonic seizures. CBZ was discontinued because we thought it would exacerbate the myoclonic seizures he was experiencing. The myoclonic seizures disappeared, and the addition of ethosuximide (ESM) completely resolved his tonic seizures. In this patient, the onset of tonic seizures was characterized by clusters, but because myoclonic seizures were not prominent initially, we were unable to diagnose Doose syndrome early. However, ESM proved remarkably effective, and the patient has remained seizure free.

日医大医会誌 2023; 19(2), 171-176

Key words
Doose syndrome, myoclonic atonic epilepsy, myoclonic seizure, carbamazepine, ethosuximide

Correspondence to
Eiji Otaka, Department of Pediatrics, Nippon Medical School, Musashi Kosugi Hospital, 1-396 Kosugi-cho, Nakahara-ku, Kawasaki, Kanagawa 211-8533, Japan
E-mail：e-otaka@nms.ac.jp

受付：2023年1月30日　受理：2023年2月24日