糖原病1a 型に破裂脳動脈瘤を合併した1 例  [in Japanese] Ruptured cerebral aneurysm complicated by glycogen storage disease type 1a: a case report  [in Japanese]

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Author(s)

    • 金城 雄太 Kaneshiro Yuta
    • 地域医療機能推進機構星ヶ丘医療センター脳神経外科 Department of Neurosurgery, Japan Community Health Care Organization, Hoshigaoka Medical Center
    • 早崎 浩司 Hayasaki Koji
    • 地域医療機能推進機構星ヶ丘医療センター脳神経外科 Department of Neurosurgery, Japan Community Health Care Organization, Hoshigaoka Medical Center
    • 大畑 建治 Ohata Kenji
    • 大阪市立大学大学院医学研究科脳神経外科学 Department of Neurosurgery, Osaka City University Graduate School of Medicine

Abstract

<p>糖原病1a 型に破裂脳動脈瘤を合併した稀な1 例を報告する.症例は34 歳女性,20 歳時に糖原病1a 型と診断された.意識障害と左片麻痺にてくも膜下出血を発症した.右中大脳動脈分岐部に3 mm の囊状動脈瘤を認め,発症当日に脳動脈瘤クリッピング術を行った.手術時に強い脳腫脹を認め外減圧を併用した.周術期は血糖や代謝性アシドーシスの補正に留意し全身管理を行った.意識障害,左片麻痺が遷延したが徐々に改善し発症3 カ月後には杖歩行が可能となった.糖原病2型と脳動脈瘤を含む脳血管異常の合併は報告されているが,糖原病1a 型と脳動脈瘤の合併の報告は渉猟したかぎり認められなかった.脳動脈瘤の成因に関して糖原病1a 型に起因する脂質異常や高尿酸血症による動脈硬化性変化,炎症性機序の関与が考えられた.強い脳浮腫は糖原病1a 型に伴う乳酸アシドーシスが関与した可能性も推察され,周術期の血糖保持,アシドーシス補正が重要と考えられた.</p>

<p>In this article, a rare occurrence of a ruptured cerebral aneurysm complicated by glycogen storage disease (GSD) type 1a has been reported. A 34-year-old woman who suffered from GSD type 1a presented with a sudden disturbance in consciousness and left hemiparesis presented to the hospital. The computed tomography (CT) imaging of the patient revealed subarachnoid hemorrhage due to the rupture of a right middle cerebral artery saccular aneurysm. Neck clipping of the ruptured aneurysm was performed on the day of symptom onset. As an excessive brain swelling was encountered during the surgery, the external decompression was performed. During and post-surgery, intensive medical treatment was provided, especially for preventing hypoglycemia and metabolic (lactic) acidosis. The disturbance in consciousness and left hemiparesis gradually improved. Three months after the surgery, the patient was able to walk with a cane. GSD type 2 is known to manifest as cerebral arterial disorders, including aneurysms. However, to the best of our knowledge, there are no reports on concurrent GSD type 1a and cerebral aneurysms in the literature. Regarding the pathogenesis of the cerebral aneurysm in this case, the authors speculate that hyperlipidemia and hyperuricemia associated with GSD type 1a may have contributed to the formation of the aneurysm through atherosclerosis or inflammation in the arterial wall. The lactic acidosis during and after the clipping surgery may have contributed to excessive brain edema. Providing intensive care to control blood glucose levels and to avoid metabolic acidosis is thought to be important for preventing secondary cerebral damages, especially in the perioperative period.</p>

Journal

  • Nosotchu

    Nosotchu 39(4), 287-291, 2017

    The Japan Stroke Society

Codes

  • NII Article ID (NAID)
    130005858957
  • Text Lang
    JPN
  • ISSN
    0912-0726
  • Data Source
    J-STAGE 
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