Neurological Surgery 脳神経外科36巻5号

2008年05月発行

総説

限局性皮質形成異常のてんかん原性に関する病態生理学的研究

著者： 程塚明¹ 田中達也¹

所属機関： ¹旭川医科大学脳神経外科

ページ範囲：P.385 - P.391

Ⅰ．はじめに

　近年，難治性てんかん症例に対して，積極的にてんかん焦点部切除等の外科的治療が施行されるようになってきた．その切除標本の病理組織学的所見より，難治性てんかん症例において，限局性皮質形成異常（FCD）などの種々の脳形成異常が病因に挙げられるようになってきた^{1,2,6,21,25,35,36)}．また近年，脳形成異常は，malformation of cortical development（MCD）という1つの概念でまとめて論じられるようになり，その分類では，proliferationの異常，migrationの異常，organizationの異常の3つが形成異常の基本として細分化されている^16,26,28)．FCDは，神経細胞の増殖過程の異常を基盤としているが，その後の遊走および皮質層構築にも異常を伴い，上記の3つの異常をすべて含んでいる^13,15,37)．

　FCDにおけるてんかん原性については，近年は病理組織学的検討などにおいて，てんかん抑制系であるGABA（gamma-aminobutyric acid）受容体の異常，興奮性アミノ酸であるグルタミン酸の受容体であるNMDA（N-methyl-D-aspartic acid）受容体等の関与も提唱されているが，未だ議論の分かれるところである^{3,4,7,8,15,20,22,29)}．

　われわれはこれまで，てんかん焦点部切除術において，術中皮質脳波検査（ECoG）等にて正確なてんかん焦点部の同定とその完全な摘出に努めてきたが，FCD等の種々の脳形成異常症例においては，病変部だけでなく，その周囲からもてんかん波形の得られる症例が認められてきた．本研究では，FCDとてんかんについて概説するとともに，新たにわれわれの作成したFCDモデル，およびてんかん焦点部摘出術を施行したFCD臨床例において，電気生理学的および病理組織学的検討を行い，そのてんかん原性につき考察を行った．

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