もやもや病の病態解析  [in Japanese] Pathophysiology of Moyamoya Disease  [in Japanese]

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Author(s)

    • 徳永 浩司 Tokunaga Koji
    • 岡山大学大学院脳神経外科 Department of Neurological Surgery, Okayama University Graduate School of Medicine
    • 杉生 憲志 Sugiu Kenji
    • 岡山大学大学院脳神経外科 Department of Neurological Surgery, Okayama University Graduate School of Medicine
    • 伊達 勲 Date Isao
    • 岡山大学大学院脳神経外科 Department of Neurological Surgery, Okayama University Graduate School of Medicine

Abstract

 もやもや病の病態解析において血管構築と遺伝子の観点から最新の知見を紹介する. 血管構築においては前方循環と外頚動脈系, 前方循環と後方循環の間にそれぞれinteractionが存在する. 前者のinteractionは「鈴木の分類」に代表され, 病態解析の中心的役割を担ってきた. 後者のinteractionは虚血重症化に関与し, 小児と成人で異なるパターンが存在する. 遺伝的要因においてはRNF213を中心とした遺伝子解析はもやもや病の病態解析に新たな局面を提供しており, 予後や治療効果の予測に応用できる可能性がある. 遺伝子治療は基礎実験の段階であるが, 血行再建術を補助する方法として期待される.

  In this article, we introduce cutting-edge knowledge pertaining to the pathophysiology of moyamoya disease from the viewpoint of angioarchitecture and genetic analysis. A complementary relationship among the anterior circulation (AC), the posterior circulation (PC) and the external carotid artery (ECA) system is involved in the pathophysiology of moyamoya disease. Suzuki's grading is representative of the interaction between the AC and ECA, and it has traditionally played a leading role in evaluating disease progression. The interaction between the AC and PC is related to hemodynamic ischemia. There is a difference between pediatric and adult moyamoya patients in the interaction between the AC and PC. The analysis of the RNF213 gene is noteworthy as a new approach to understanding the pathophysiology of moyamoya disease. The prognosis and responsiveness to revascularization surgery can be predicted on the basis of genetic analysis. Although gene therapy for moyamoya disease is only now just in the basic experimental studies stage, it can be expected to emerge as an assisting modality for indirect revascularization surgery.

Journal

  • Japanese Journal of Neurosurgery

    Japanese Journal of Neurosurgery 24(4), 239-243, 2015

    The Japanese Congress of Neurological Surgeons

Codes

  • NII Article ID (NAID)
    130005066023
  • Text Lang
    JPN
  • ISSN
    0917-950X
  • Data Source
    J-STAGE 
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