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Neurological Surgery No Shinkei Geka Volume 48, Issue 5 （May 2020）

Neurological Surgery 脳神経外科 48巻5号（2020年5月発行）

Japanese English

症例

産褥期に皮質性くも膜下出血と可逆性脳血管攣縮症候群を合併したaplastic or twig-like middle cerebral arteryの若年女性例 Aplastic or Twig-like Middle Cerebral Artery with Cortical Subarachnoid Hemorrhage and Reversible Cerebral Vasoconstriction Syndrome during the Postpartum Period in a Juvenile Female:A Case Report 松尾彩香 ¹ , 日宇健 ¹ , 伊藤健大 ¹ , 森塚倫也 ¹ , 本田和也 ¹ , 川原一郎 ¹ , 小野智憲 ¹ , 原口渉 ¹ , 牛島隆二郎 ¹ , 堤圭介 ¹ Ayaka MATSUO ¹ , Takeshi HIU ¹ , Takehiro ITO ¹ , Tomoya MORITSUKA ¹ , Kazuya HONDA ¹ , Ichiro KAWAHARA ¹ , Tomonori ONO ¹ , Wataru HARAGUCHI ¹ , Ryujiro USHIJIMA ¹ , Keisuke TSUTSUMI ¹ ¹国立病院機構長崎医療センター脳神経外科 ¹Department of Neurosurgery, National Hospital Organization Nagasaki Medical Center キーワード： twig-like middle cerebral artery , incidental , adolescent case , cortical subarachnoid hemorrhage , reversible cerebral vasoconstriction syndrome Keyword: twig-like middle cerebral artery , incidental , adolescent case , cortical subarachnoid hemorrhage , reversible cerebral vasoconstriction syndrome pp.435-444

発行日 2020年5月10日 Published Date 2020/5/10

DOI https://doi.org/10.11477/mf.1436204206

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Abstract 文献概要
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参考文献 Reference

Ⅰ．はじめに

　発生途上の中大脳動脈M1部は網状側副血管（twig-like network：TLN）と呼ばれる網状構造として存在し，その癒合と退縮によって正常M1へと発達する²⁶⁾．この過程が未知の要因により乱された場合にM1形成不全（無形成〜低形成）が起こり，これを補うためにTLNを有する破格側副血行路が遺残すると考えられている^4,19)．M1形成不全状態やTLNの遺残状況は種々の程度・構造で混在するため，包括した概念として，aplastic or twig-like middle cerebral artery（Ap/T-MCA）という診断名が提唱された³¹⁾．このように，Ap/T-MCAは発生異常として捉えられているが，若年者の報告例は少ない^21,35)．

　今回われわれは，産褥期に皮質性くも膜下出血（cortical subarachnoid hemorrhage：cSAH）と可逆性脳血管攣縮症候群（reversible cerebral vasoconstriction syndrome：RCVS）を合併した若年女性例を経験した．本例の病態と若年Ap/T-MCA症例（20歳未満）の臨床像を中心に，文献的考察を加えて報告する．

　A 17-year-old female in a lethargic state with mild dysarthria was transferred to our hospital after experiencing a generalized tonic seizure immediately after giving birth. Head CT showed a cortical subarachnoid hemorrhage（cSAH）in the left frontoparietal convexity. Three-dimensional rotational angiography（3D-RA）revealed multifocal narrowing of the cortical branches of the left middle cerebral artery（MCA）and severe stenosis of the left M1 segment with plexiform collateral networks, suggesting the presence of reversible vasoconstriction syndrome（RCVS）and aplastic or twig-like MCA（Ap/T-MCA). When 3D-RA was repeated on day 17, the narrowing of the cortical artery had resolved, and a new constriction of more proximal blood vessels was observed. The arterial spasm disappeared within 3 months, confirming the diagnosis of RCVS and Ap/T-MCA.

　Although non-aneurysmal SAH due to Ap/T-MCA is extremely rare, RCVS often complicates cSAH in the frontal/parietal region. It is suggested that RCVS triggers cSAH in the presence of incidental Ap/T-MCA.

　Ap/T-MCA is thought to be caused by developmental abnormalities during the embryonic period, but only 11 cases in children or adolescents have been reported. This suggests that there are a considerable number of asymptomatic young patients whose condition has not been detected. The majority of patients with Ap/T-MCA are from East Asia, suggesting that racial and genetic background differences are a factor. As this anomaly is more likely to present as a stroke in adulthood, long-term follow-up is recommended if it is found at a young age. There is no evidence that revascularization is effective in preventing stroke. Further studies are needed on how to manage this condition appropriately.