CAJ | 학술논문

目的：探讨烟雾病合并Graves病(毒性弥漫性甲状腺肿)的临床特征、发病机制、手术及药物治疗的效果。方法回顾性分析4例成人烟雾病合Graves病患者的临床资料，所有患者均符合烟雾病及Graves病的诊断标准。3例患者在甲亢稳定1个月后，1例在甲状腺功能未完全正常时行直接间接联合血管重建术。结果①4例患者中，3例为女性，1例男性。平均年龄32.3岁（27-42岁）。②4例患者中，3例表现为脑梗塞，1例表现为频发TIA。影像学DSA证实2例为单侧烟雾病，2例为双侧烟雾病；MR均可见脑梗塞灶。③对4例患者的随访时间为6-18个月，4患者经治疗后，神经功能症状均减轻或消失，无新发神经功能障碍。SPECT提示脑缺血灶较术前明显改善，CTA提示吻合口通畅。无手术并发症。结论烟雾病合并Graves病以女性患者为多，临床症状主要表现为脑缺血发作或脑梗死。控制甲亢症状后，行手术治疗能够建立有效的侧支循环，改善临床症状。手术时机须根据甲状腺功能及烟雾病症状宜早实施。
목적：탐토연무병합병Graves병(독성미만성갑상선종)적림상특정、발병궤제、수술급약물치료적효과。방법회고성분석4례성인연무병합Graves병환자적림상자료，소유환자균부합연무병급Graves병적진단표준。3례환자재갑항은정1개월후，1례재갑상선공능미완전정상시행직접간접연합혈관중건술。결과①4례환자중，3례위녀성，1례남성。평균년령32.3세（27-42세）。②4례환자중，3례표현위뇌경새，1례표현위빈발TIA。영상학DSA증실2례위단측연무병，2례위쌍측연무병；MR균가견뇌경새조。③대4례환자적수방시간위6-18개월，4환자경치료후，신경공능증상균감경혹소실，무신발신경공능장애。SPECT제시뇌결혈조교술전명현개선，CTA제시문합구통창。무수술병발증。결론연무병합병Graves병이녀성환자위다，림상증상주요표현위뇌결혈발작혹뇌경사。공제갑항증상후，행수술치료능구건립유효적측지순배，개선림상증상。수술시궤수근거갑상선공능급연무병증상의조실시。
Objective To investigate the clinical features, operation and drug treatment effect of Moyamoya disease complicated with Graves disease (GD). Methods A retrospective analysis was done to 4 patients' clinical data of adult Moyamoya disease complicated with Graves disease, all patients were diagnosed Moyamoya disease and Graves disease. Direct/indirect combined vascular reconstruction were performed in 4patients, 3 patients complicated with hyperthyroidism were stable for 1 months, 1 patient was not completely normal in thyroid function. Results①3 patients were female, 1 male cases of the 4 patients. The average age was 32.3 years (27-42 years). ②Of the 4 patients, 3 cases showed cerebral infarction, 1 cases showed frequent TIA. Digital subtraction angiography confirmed 2 cases of unilateral moyamoya disease, 2 cases were bilateral moyamoya disease; magnetic resonance showed cerebral infarction. ③4 patients were followed up for 6-18 months, 4 patients were relieved or disappeared of neurological symptoms after treatment, no new neurological dysfunction appearred. SPECT showed the focal brain ischemia significantly improved compared with preoperative, CTA showed prompt anastomosis. No operation complication occurred. Conclusion Moyamoya disease complicated with Graves disease is more common in female, with cerebral infarction or cerebral ischemic attack as the main clinical symptoms.Surgical operation can establish effective collateral circulation and improve the clinical symptoms after the hyperthyroidism symptoms were controlled. Timing of operation should be implemented earlier according to the thyroid function and symptoms of moyamoya disease.