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  1. Dokkyo Journal of Medical Sciences
  2. 34(1) 2007

A Case of Schizencephaly Type I Associated with West Syndrome that Responded to ACTH Treatment

https://dmu.repo.nii.ac.jp/records/531
https://dmu.repo.nii.ac.jp/records/531
9128343b-e6a5-422a-ba26-cb9454b16f50
名前 / ファイル ライセンス アクション
KJ00005058607.pdf KJ00005058607.pdf (594.4 kB)
Item type [ELS]学術雑誌論文 / Journal Article(1)
公開日 2017-05-24
タイトル
言語 en
タイトル A Case of Schizencephaly Type I Associated with West Syndrome that Responded to ACTH Treatment
言語
言語 eng
キーワード
言語 en
主題 West syndrome
キーワード
言語 en
主題 schizencephaly
キーワード
言語 en
主題 ACTH
キーワード
言語 en
主題 infantile spasm
キーワード
言語 en
主題 magnetic resonance imaging
資源タイプ
資源タイプ識別子 http://purl.org/coar/resource_type/c_6501
資源タイプ journal article
雑誌書誌ID
収録物識別子 AA00629581
論文名よみ
タイトル A Case of Schizencephaly Type I Associated with West Syndrome that Responded to ACTH Treatment
著者 Imataka, George

× Imataka, George

WEKO 2168

en Imataka, George

Search repository
Kuwashima, Shigeko

× Kuwashima, Shigeko

WEKO 2169

en Kuwashima, Shigeko

Search repository
Hashimoto, Teisuke

× Hashimoto, Teisuke

WEKO 2170

en Hashimoto, Teisuke

Search repository
Yamanouchi, Hideo

× Yamanouchi, Hideo

WEKO 2171

en Yamanouchi, Hideo

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Arisaka, Osamu

× Arisaka, Osamu

WEKO 2172

en Arisaka, Osamu

Search repository
著者所属(英)
en
Department of Pediatrics, Dokkyo Medical University Tochigi
著者所属(英)
en
Department of Radiology, Dokkyo Medical University Tochigi
著者所属(英)
en
Department of Radiology, Dokkyo Medical University Tochigi
著者所属(英)
en
Department of Pediatrics, Dokkyo Medical University Tochigi
著者所属(英)
en
Department of Pediatrics, Dokkyo Medical University Tochigi
記事種別(日)
内容記述タイプ Other
内容記述 症例報告
記事種別(英)
内容記述タイプ Other
内容記述 Case Report
抄録(英)
内容記述タイプ Other
内容記述 Although schizencephaly is known to be complicated by various types of epilepsy, complication by West syndrome is rare. We encountered a boy with West syndrome accompanied by schizencephaly. There was no abnormality in the birth history. After birth, the right upper and lower limb movements were less frequent and weaker than those on the left side. The neck was stabilized at 4 months of age. At 5 months of age, the boy developed infantile spasms, and hypsarrythmia was noted on EEG. Brain MRI detected schizencephaly fused with the cerebral wall in the left temporal to parietal region, and schizencephaly type I was diagnosed. From the clinical features of the seizures and EEG findings, he was diagnosed with symptomatic West syndrome. ACTH therapy was performed at 0.02 mg/kg day, and hypsarrythmia was improved on EEG on day 21 and the seizures disappeared. Seizures have been controlled to left facial partial seizures by anticonvulsive monotherapy with carbamazepine thereafter. Brain MRI after ACTH therapy detected cerebral cortical atrophy. On ^<99m>Tc ECD-SPECT performed at 7 months, a decreased cerebral blood flow and defect were noted in a consistent shape with the schizencephaly on the left side. Spastic paralysis of the right upper and lower limbs remains. The right ankle joint shows foot-drop, but the patient can walk with an orthosis attachment. The patient was enrolled in a general class in primary school, and mental retardation is mild. In the Discussion, the characteristics of epilepsy in schizencephaly and symptomatic West syndrome, and the cause of schizencephaly are described with a review of the literature.
書誌情報 Dokkyo journal of medical sciences

巻 34, 号 1, p. 57-61, 発行日 2007-03-01
ISSN
収録物識別子 03855023
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