The indications for surgical intervention for epilepsy associated with chromosomal abnormalities remain controversial. We report a 13-year-old girl with trisomy 21 and refractory epilepsy. The patient developed West syndrome at the age of 5 months. The seizures were refractory to multiple antiepileptic drugs, adrenocorticotropic hormone therapy, and a ketogenic diet. The patient developed Lennox-Gastaut syndrome at the age of 7 years. Frequent daily seizures including several sound-induced and tonic seizures impeded daily living of the patient and her caregivers. For alleviation of these seizures, the patient underwent total

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。

To describe the far-anterior interhemispheric transcallosal approach for the treatment of a central neurocytoma at the roof of the lateral ventricle. In comparison to the view obtained during the usual anterior transcallosal approach, the far-anterior approach allowed for a higher view of the lateral ventricle to be obtained without further injury or retraction of the

Hemispherotomy is a radical treatment for drug-resistant epilepsy that targets developmental, acquired, and progressive diseases with widespread epileptogenic regions in one cerebral hemisphere. Currently, two main approaches are utilized after repeated improvements: lateral and vertical approaches. With the lateral approach, the surgical field is wide, and the approach to the lateral ventricle is relatively easy. On the other hand, the vertical approach has the advantage of reducing intraoperative bleeding and operating time as the resection line of the radial fiber is short, and understanding the three-dimensional anatomy is relatively easy. The lateral approach is generally used for atrophic lesions, whereas the vertical approach is for hypertrophic lesions. Hemispherotomy is expected to not only suppress epileptic seizures but also improve psychomotor development by protecting the unaffected cerebral hemisphere. However, this method is one of the most invasive surgeries in epilepsy surgery, and it is important to fully consider its indications. Furthermore, understanding the neural fiber pathway is important for actual surgery.

This study investigated the number of epilepsy surgeries performed over time in Japan, and conducted a questionnaire survey of the Japan Neurosurgical Society (JNS) training program core hospitals to determine the current status and future objectives of surgical therapies and epilepsy training programs for physicians in Japan. This article presents part of a presentation delivered as a presidential address at the 44th Annual Meeting of the Epilepsy Surgery Society of Japan held in January 2021. The number of epilepsy surgeries performed per year has increased in Japan since 2011 to around 1,200 annually between 2015 and 2018. The questionnaire survey showed that 50% of the responding hospitals performed epilepsy surgery and 29% had an epilepsy center, and that these hospitals provided senior residents with education regarding epilepsy surgery. The presence of an epilepsy center in a hospital was positively correlated with the availability of long-term video electroencephalography monitoring beds as well as the number of epilepsy surgeries performed at the hospital. In regions with no medical facilities offering specialized surgical therapies for epilepsy, the JNS training program core hospitals may help improve epilepsy diagnosis and treatment. They may also increase the number of safe and effective surgeries by establishing epilepsy centers that can perform long-term video electroencephalography monitoring, providing junior neurosurgeons with training regarding epilepsy, and playing a core role in surgical therapies for epilepsy in tertiary medical areas in close cooperation with neighboring medical facilities.

 難治性てんかんに対する外科手術は有用な治療手段である. 非侵襲的検査において焦点の局在同定が困難な場合, 頭蓋内電極を用いた精査が行われる. 従来は硬膜下電極を用いることが一般的であったが, 近年では定位的手法で挿入した頭蓋内電極で焦点診断を行うことが諸外国で増加している. てんかん焦点が局在している場合, より低侵襲な手術が望ましい. 一方で, 広範囲のてんかんネットワークが発作に関与している場合, 脳梁や脳葉の離断を概念とした手術を考慮する必要がある. 本邦未導入であるが, 近年では局所の凝固術や, 視床前核への深部脳刺激, 反応性発作起始領域刺激などの新規治療も行われるようになってきている.