Corpus callosotomy is considered the most effective procedure for preventing tonic, atonic, and other seizures causing a fall. This study reports a case of a 6-year-old female, diagnosed with trisomy 21 following birth, wherein corpus callosotomy was effective for the treatment of atypical absence status epilepticus. She developed West syndrome at eight months of age. At 5 years, she experienced multiple seizure types, including tonic, myoclonic, and atypical absence seizures. She was hospitalized at 6 years 0 months, because of her inability to walk and difficulty in eating with the increase in atypical absence seizures to more than 100 times a day. The condition was intractable and corpus callosotomy was performed at 6 years and 1 month. The postoperative electroencephalogram revealed generalized spike-wave complexes localized to the right frontal area. Two years have passed since, and her seizures have disappeared. There have been several reports regarding the effects of corpus callosotomy on atypical absence seizures. This case suggests that corpus callosotomy may be a prominent treatment option for intractable atypical absence seizures.
"},{"@language":"ja","@value":"脳梁離断術はてんかん性スパズム、強直発作、脱力発作などの転倒する発作で最も有効とされる。今回、非定型欠神発作重積に脳梁離断術が有効だった1例を経験したので報告する。症例は6歳女児。生後Down症候群と診断された。8カ月時にWest症候群を発症した。5歳時から強直発作、ミオクロニー発作、非定型欠神発作などの多彩な発作を認めるようになり、Lennox-Gastaut syndrome spectrumと診断した。6歳0カ月時には最大で1日100回以上の非定型欠神発作が出現して、脳波検査では発作時、発作間欠時脳波ともに全般性変化を呈した。難治に経過し、薬剤治療による欠神発作重積の抑制は困難と判断して6歳1カ月時に脳梁離断術を行った。手術直後から各種発作は消失した。術後の脳波では全般性棘徐波が右前頭部に局在化した。脳梁を介した神経興奮の拡がりが脳梁離断術によって抑制されたことによって、全般発作である非定型欠神発作が抑えられた可能性を考えた。本症例の経験から、特に欠神発作重積が薬剤抵抗性で退行を呈するなどの緊急性を要する症例では、脳梁離断術の適応を積極的に検討すべきと考えた。
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University"}]},{"@id":"https://cir.nii.ac.jp/crid/1410013485542863877","@type":"Researcher","foaf:name":[{"@language":"en","@value":"Matsufuji Mayumi"},{"@language":"ja","@value":"松藤 まゆみ"}],"jpcoar:affiliationName":[{"@language":"ja","@value":"独立行政法人国立病院機構南九州病院小児科"},{"@language":"en","@value":"Division of Pediatrics, National Hospital Organization Minamikyushu Hospital"}]},{"@id":"https://cir.nii.ac.jp/crid/1410013485542863876","@type":"Researcher","foaf:name":[{"@language":"en","@value":"Sano Nozomi"},{"@language":"ja","@value":"佐野 のぞみ"}],"jpcoar:affiliationName":[{"@language":"ja","@value":"社会医療法人童仁会池田病院小児科"},{"@language":"en","@value":"Ikeda Hospital"}]},{"@id":"https://cir.nii.ac.jp/crid/1410013485542863878","@type":"Researcher","foaf:name":[{"@language":"en","@value":"Sato Masanori"},{"@language":"ja","@value":"佐藤 雅紀"}],"jpcoar:affiliationName":[{"@language":"ja","@value":"鹿児島大学大学院医歯学総合研究科脳神経外科学"},{"@language":"en","@value":"Department of Neurosurgery, Graduate School of Medical and Dental Sciences, Kagoshima University"}]},{"@id":"https://cir.nii.ac.jp/crid/1410013485542863873","@type":"Researcher","foaf:name":[{"@language":"en","@value":"Hosoyama Hiroshi"},{"@language":"ja","@value":"細山 浩史"}],"jpcoar:affiliationName":[{"@language":"en","@value":"Department of Neurosurgery, Kagoshima City Hospital"},{"@language":"ja","@value":"鹿児島市立病院脳神経外科"}]},{"@id":"https://cir.nii.ac.jp/crid/1410013485542863874","@type":"Researcher","foaf:name":[{"@language":"en","@value":"Hanaya Ryosuke"},{"@language":"ja","@value":"花谷 亮典"}],"jpcoar:affiliationName":[{"@language":"ja","@value":"鹿児島大学大学院医歯学総合研究科脳神経外科学"},{"@language":"en","@value":"Department of Neurosurgery, Graduate School of Medical and Dental Sciences, Kagoshima University"}]}],"publication":{"publicationIdentifier":[{"@type":"PISSN","@value":"09120890"},{"@type":"LISSN","@value":"09120890"},{"@type":"EISSN","@value":"13475509"}],"prism:publicationName":[{"@language":"en","@value":"Journal of the Japan Epilepsy Society"},{"@language":"ja","@value":"てんかん研究"},{"@language":"en","@value":"JJES"},{"@language":"en","@value":"J.Jpn.Epil.Soc."},{"@language":"ja","@value":"てんかん研究"}],"dc:publisher":[{"@language":"en","@value":"JAPAN EPILEPSY SOCIETY"},{"@language":"ja","@value":"一般社団法人 日本てんかん学会"}],"prism:publicationDate":"2023-01-30","prism:volume":"40","prism:number":"3","prism:startingPage":"541","prism:endingPage":"547"},"reviewed":"false","url":[{"@id":"https://www.jstage.jst.go.jp/article/jjes/40/3/40_541/_pdf"},{"@id":"https://search.jamas.or.jp/link/ui/2023125718"}],"availableAt":"2023-01-30","foaf:topic":[{"@id":"https://cir.nii.ac.jp/all?q=atypical%20absence","dc:title":"atypical absence"},{"@id":"https://cir.nii.ac.jp/all?q=status%20epilepticus","dc:title":"status epilepticus"},{"@id":"https://cir.nii.ac.jp/all?q=corpus%20callosotomy","dc:title":"corpus callosotomy"},{"@id":"https://cir.nii.ac.jp/all?q=West%20syndrome","dc:title":"West 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