Neurological Surgery 脳神経外科43巻6号

2015年06月発行

連載 脳卒中専門医に必要な基本的知識

（11）もやもや病に対する治療方針

著者： 藤村幹¹ 冨永悌二¹

所属機関： ¹東北大学脳神経外科

ページ範囲：P.557 - P.565

Ⅰ．はじめに
　もやもや病は本邦で初めて報告され，1969年にSuzukiらによりmoyamoya diseaseと命名された原因不明の疾患である⁴¹⁾．両側内頚動脈終末部，前および中大脳動脈近位部の進行性狭窄・閉塞と，その付近の異常血管網の発達といった脳血管像上の特徴で定義される^14,27,41)．本疾患は日本人をはじめとする東洋人に多くみられるが，本邦の最新の疫学調査においても日本国内の患者数は約7,700人，発生率は人口10万人あたり0.54人と推定されている稀な疾患である²⁸⁾．男女比は約1：1.8と女性に多いことが知られており，発症の年齢分布は10歳以下を中心とする若年型と30〜40歳を中心とする成人型の二峰性を示す²⁸⁾．幼小児期ならびに若年発症の一過性脳虚血発作，脳梗塞，そして脳出血の原因として重要な疾患である．
　虚血発症例に対しては頭蓋外・頭蓋内血行再建術（EC-ICバイパス）が有効で，脳血流の改善による虚血発作の抑制，脳梗塞への進行の予防が期待できる^{1,3,16,20,25,34,40)}．出血発症例における再出血も重要な予後増悪因子として知られているが，血行再建術の再出血予防効果については多施設間共同研究（Japan Adult Moyamoya trial：JAM trial）による検証が行われ，EC-ICバイパスの再出血予防効果も示唆された³¹⁾．本稿ではもやもや病の基礎病態，手術適応そして手術手技と周術期管理について概説する．

参考文献

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