甲状腺功能亢进症合并烟雾综合征

Moyamoya Syndrome in Graves’ Disease

目的分析和探讨经颅多普勒超声(TCD)筛查的甲状腺功能亢进症(甲亢)合并烟雾综合征的临床和血管病变特征。方法总结北京协和医院和解放军307医院2000～2007年经TCD筛查诊断烟雾综合征患者13例,8例经磁共振血管成像(MRA)、5例经数字减影血管造影(DSA)明确诊断;所有患者经协和医院内分泌科确诊为甲亢,且甲状腺球蛋白抗体(TGAb)和髓过氧化物酶抗体(TPOAb)高,除外血管狭窄的其他常见原因。结果13例患者中,男1例,女12例;11例表现为缺血性卒中(2例合并不自主运动),1例仅表现为不自主运动,1例以头晕为临床主诉,后2例经TCD筛查发现血管狭窄。血管病变分布:11例为典型的颈内动脉终末端(TICA)、大脑中动脉(MCA)、大脑前动脉(ACA)狭窄或闭塞,1例为颈内动脉虹吸段狭窄,1例为双侧颈内动脉起始严重狭窄或闭塞。2例患者同时合并大脑后动脉(PCA)狭窄。随访发现,1例患者因甲亢控制不佳复查TCD血管病变加重;而1例甲状腺功能稳定,缺血临床症状明显改善。4例患者行颞浅动脉-大脑中动脉(MCA)血管融通术,术后缺血发作减少。结论TCD的临床应用使越来越多的甲亢合并烟雾综合征得以诊断;烟雾综合征患者常规行甲状腺功能筛查十分必要;血管成形术可能是预防缺血性卒中的有效方法之一。

Objective To analyze the clinical manifestation and intracranial lesions in patients concomitant with Graves' disease (GD) and moyamoya syndrome(MMS) screened by transcranial Doppler (TCD). Methods We screened MMS by TCD for patients with ischemic stroke from 2000 to 2007 and these patients were diagnosed for MMS by digital subtract angiography (DSA) or magnetic resonance angiography(MRA). All the patients were diagnosed for GD with high level of thyroglobulin antibody(TGAb) and thyroid peroxidase autoantibody(TPOAb) at the same time. Results A total of 13 GD patients (1 male and 12 female) concomitant with MMS were screened during 7 years. Eleven patients had ischemic stroke (two patients accompanied with chorea), while one had dizziness and one had chorea only. Eleven patients had stenosis/occlusion on bilateral terminal internal carotid arteries (TICAs) /middle cerebral arteries(MCAs) /anterior cerebral arteries(ACAs), one patient with unilateral siphon of internal carotid artery(ICA) lesions, and the remaining patient had occlusion of proximal ICA in both sides. In all the patients as follow-up, we found vascular lesions aggravated by TCD in one patient with GD uncontrolled and another patient with improvement of cerebral ischemic symptom and stable thyroid function. Four patients underwent bilateral superfi cial temporal artery(STA)-MCA anastomosis after normalization of her hormonal conditions in 307 hospital of people's liberation army(PLA). The neurological condition was improved since revascularization procedure. Conclusion According to the TCD screening, we find MMS associated with GD may not rare. Thyroid function test should be performed for the patients who have MMS without risk factors of atherosclerosis. Revascularization is thought to be an appropriate management for prevention further ischemic stroke.