2026-03-12T20:33:37Z https://dmu.repo.nii.ac.jp/oai

oai:dmu.repo.nii.ac.jp:02000309 2025-07-14T02:04:17Z 81:1724903586492

フェノバルビタールが奏功した乳児てんかん性スパズム症候群の 2 か月女児 A case Report of a 2-month-old Girl with Infantile Epileptic Spasm Syndrome Who Exhibited a Favorable Response to Phenobarbital 梶谷, さゆり 今高, 城治 斉田, 沙佑美 吉原, 伸弥 高栁, 文貴 藤田, 雄治 白石, 秀明 乳児てんかん性スパズム症候群 大田原症候群 West症候群 Infantile epileptic spasms syndrome Ohtahara syndrome West syndrome 症例は生後 2 か月女児．入眠中 15～20 秒の周期で両上肢を瞬時に伸展し回外させるシリーズ形成性スパズムを認めた．脳波検査で異常放電は認めなかったが，フェノバルビタール（Phenobarbital：PB）投与開始後，発作は消失した．その後 PB の血中濃度依存性に発作が増減した．経過中に明らかなてんかん性異常波は認めず，PB 投与を継続し，生後 3 か月に退院した．乳児早期のシリーズ形成性スパズムは大田原症候群，乳児ミオクロニーてんかん，West 症候群との鑑別が重要である．原因が明らかでない乳児てんかん性スパズム症候群の初期治療に対し，PB を投与しつつ臨床経過を観察し，鑑別を進めることが重要と考えられた Early infantile series-forming spasms are a form of epilepsy that is difficult to differentiate from other forms of infantile epilepsy, including Ohtahara and West syndrome and infantile myoclonic epilepsy. In this report, we present the case of a 2-month-old girl who was admitted to our hospital with recurring seizures, characterized by rapid extension of both upper limbs during sleep. The child exhibited recurrent episodes of series-forming spasms, lasting 15-20 seconds, involving simultaneous extension and rotation of both upper limbs. She was born without pseudopnea during a normal delivery. Sleep video electroencephalography（EEG）failed to establish Ohtahara or West syndrome, as no irregular discharge was identified. Treatment was initiated by the intravenous administration of phenobarbital（PB）. As the frequency of seizures increased and decreased in a concentration-dependent manner, the PB concentration in the blood was maintained at a consistently high level, resulting in cessation of seizures. Periodic EEG did not reveal any notable irregular epileptic waves, and the patient’s development was deemed satisfactory at the age of 6 months following continuous outpatient treatment. For the initial treatment of infantile epileptic spasm syndrome of an unknown cause, it is essential to observe the clinical course with simultaneous differentiation while administering PB. journal article 獨協医学会 2024-07-25 VoR application/pdf Dokkyo Journal of Medical Sciences 1 44 47 AA00629581 0385-5023 https://dmu.repo.nii.ac.jp/record/2000309/files/DKMJ-51-1-9.pdf https://dmu.repo.nii.ac.jp/records/2000309 jpn 2025054463