烟雾病的研究现状及进展

烟雾病（MMD）是颈内动脉颅内段及其属支进行性狭窄、闭塞及脑底出现异常扩张侧枝循环网所致的脑出血性或缺血性疾病。此病发病年龄呈双峰型。成年患者经常表现为出血症状,而儿童多表现为短暂缺血发作或中风。血管造影是诊断的金标准。外科手术是主要治疗方法,通过增加皮质血供来预防脑缺血损伤。本文就MMD的流行病特征、病因、病理及临床诊治方面的最新研究进展作一综述。     

成人烟雾病治疗的相关研究进展

烟雾病（moyamoya disease,MMD）是一种慢性进行性脑血管疾病,涉及颈内动脉末端和/或其近端分支狭窄闭塞,导致侧支血管网形成。这些变化引起脑实质慢性缺血,随后发生严重的脑血管意外。成人MMD患者在未经治疗的情况下会逐渐累及认知功能,且病死率是儿童MMD患者的2倍。由于MMD发展病因的复杂性和后果的严重性,该病的治疗尤为棘手且迫切。外科血管重建术作为MMD治疗的基石,主要分为直接血管重建术、间接血管重建术和联合血管重建术三类。考虑到间接血管重建术不能降低围手术期脑卒中的发生率,直接血管重建术通常是缺血型MMD患者治疗的首选术式。若为了预防出血型MMD患者再发出血,选择直接血管重建术或联合血管重建术能够更容易建立侧支循环,促进血运重建,达到治疗的目的。当患者术后出现新发的缺血脑卒中,则优先考虑间接血管重建术。对于血流动力学不稳定的成人MMD患者,直接血管重建术或联合血管重建术则是首要选择。由于目前无逆转MMD病情进展的特效药,内科治疗仅局限于对症治疗和围手术期的管理。临床上通常使用阿司匹林抗血小板聚集,促红细胞生成素和他汀类药物等促进侧支血管发育和地塞米松促进新生血管的形成等。远隔缺血适应训练改善MMD患者的后遗症的疗效已经得到了业界的肯定。发病机制研究的深入为MMD的诊疗手段提供更多的可能性。甲硫氨酸循环异常参与了MMD的发病,提示甲硫氨酸循环相关风险评分对烟雾病风险具有良好的预测能力。此外,内皮祖细胞移植可能成为临床上治疗MMD的新策略。 [国际神经病学神经外科学杂志, 2023, 50(3): 78-83]     

头颅CT灌注成像对烟雾病行间接血流重建术的疗效评价

目的研究探讨头颅CT灌注成像（CTP）技术对缺血型或出血型烟雾病（MMD）患者行间接血流重建术（indirect revascularisation）的总体疗效评价。方法 47例缺血性或出血卒中起病并由数字减影血管造影（DSA）证实的MMD患者,采用颅骨多点钻孔术（MBHT）、脑-颞肌贴敷术（EMS）、脑-颞肌-动脉贴敷术（EDAMS）、脑-硬脑膜-动脉贴敷术行间接血流重建术。并于术前、术后1周、术后3个月分别行头颅CT灌注成像检查,分别对脑血流量（CBF）、脑血容量（CBV）、达峰时间（TTP）进行定量和定性检查分析,并对手术前后及随访的CBF、CBV、TTP进行对比分析。结果 MMD患者术前与术后早期（1周）CTP各参数比较,额颞叶CBF、CBV变化无统计学差异,TTP显著降低,差异有统计学意义（P〈0.05）;术后3个月CTP与术后1周比较,额、颞叶rTTP、rCBV降低;差异有统计学意义（P〈0.05）。慢性缺血型MMD患者术后症状改善明显。结论 CT灌注成像作为一项快速简便的脑血流状况的检测技术,能反映脑微循环信息的功能成像,尤其是其TTP等时间参数对区分缺血程度具有很高的敏感性,能显示早期细微的缺血变化,MMD间接血流重建术后侧支血管的形成和脑血流的改善远早于造影上新生血管的形成,可以解释间接血流重建术后早期在造影可见的吻合血管出现之前就出现临床症状的明显改善。该检查有助于判断MMD脑缺血的部位和程度、选择手术侧别和手术方式,更为了解间接血流重建术后缺血程度是否改善提供较客观的评判依据。     

脑血管病变与阿尔茨海默病关系的研究进展

阿尔茨海默病(Alzheimer′s disease，AD)是一种中枢神经系统进行性的退行性病变。其病因与遗传、病毒感染、炎症、铝中毒、胆碱系统功能缺陷、细胞骨架改变等有关。近年来的流行病学及病理学研究表明，AD与血管因素有关联。血管因素不仅会造成血管性痴呆(vascular dementia，VD)，也可能参与AD的病理生理机制，     

脑小血管病研究进展

正脑小血管病是临床常见的脑血管疾病,25%的卒中及45%的痴呆由脑小血管病引起,其危害性近来愈来愈受到重视。脑小血管病的临床表现多样,可从无症状到各种神经精神症状,容易造成诊断及治疗上的混乱。近年来,脑小血管病研究已从早期的临床观察深入到神经影像、神经病理、神经生物等方面,极大的推进了对脑小血管病的认识。本文就脑小血管病的流行病学、病理生理机制、临床表现、神经影像、治疗等方面展开综述,便于临床医生进一步了解脑小血管病。1定义及流行病学脑小血管病是指各种病因影响脑内小动脉、毛细血管、     

成年型烟雾病患者血管内皮生长因子和基质金属蛋白酶-9血清水平与Suzuki分级的关系

目的 探讨成年型烟雾病( moyamoya disease,MMD)患者血管内皮生长因子(vascular endothelial growth factor,VEGF)和基质金属蛋白酶-9(matrix metalloproteinase-9,MMP-9)血清水平及其与Suzuki分级的关系.方法 从南京卒中注册系统中提取2009年4月至2011年1月间以短暂性脑缺血发作或首发卒中的52例住院或门诊复诊的成年型MMD患者.同时选取16名性别和年龄与MMD患者相配匹的健康人作为对照组.通过酶联免疫吸附分析法,比较MMD患者VEGF和MMP-9血清水平与健康对照人群间的差异;根据Suzuki 6级分级标准,将MMD患者分为不同亚组,分别分析不同亚组VEGF和MMP-9血清水平与Suzuki分级的相关性;另外,对MMD患者VEGF和MMP-9血清水平的相关性亦作了分析.结果 缺血型和出血型MMD患者VEGF血清水平分别为(289.4±69.2)pg/ml和(324.3±95.6) pg/ml,均高于健康对照组[(63.5±7.6) pg/ml;F=69.43;P ＜0.01];缺血型和出血型MMD患者MMP-9血清水平分别为(499.4±76.2)ng/ml和(531.2±100.2)ng/ml,亦均高于健康对照组[(257.1±30.7)ng/ml;F =66.023;P ＜0.01].随着Suzuki分级的升高,VEGF和MMP-9血清水平呈现升高趋势,相关系数r分别为0.879(P ＜0.01)和0.838(P ＜0.01).另外,VEGF和MMP-9之间存在正向线性相关(r=0.590,P＜0.01).结论 成年型MMD患者VEGF和MMP-9血清水平高于健康人群,提示参与了血管新生;随着患者病情的进展,VEGF和MMP-9血清水平呈现升高趋势,提示VEGF和MMP-9血清水平能反映患者的病变严重程度.     

癫(癎)与人类疱疹病毒6型感染关系研究进展

癫(癎)具有复杂的病因,对癫(癎)病因的深入了解有可能提供更具有针对性和有效的治疗,获得更好的临床效果.尽管血管畸形、脑外伤、脑肿瘤等获得性病因以及涉及多种离子通道的遗传因素等已经逐渐被人们认识,但大多数癫(癎)病因以及病理生理机制仍然远未阐明.     

12例儿童烟雾病的临床及影像研究

目的研究儿童型烟雾病（MMD）的临床及影像学特征。方法回顾性分析12例儿童型MMD的临床资料，观察其临床及影像学特点。结果本组病例女性居多，病程为反复多发．起病主要有短暂脑缺血发作、脑梗死、出血等形式，临床表现为肢体麻木、无力、头痛、智力减退等症状：数字减影血管造影（DSA）在脑底动脉环发现血管闭塞、狭窄及炯雾状毛细血管网形成，MRA对本病有一定阳性发现。结论对于儿童反复发作的脑卒中患者要考虑MMD的可能，目前仍依靠DSA确诊，MRA对本病有一定阳性发现。可考虑血管重建手术治疗。     

全瘤型垂体卒中MRI特征及相关因素分析

目的探讨全瘤型垂体卒中(PA)的MRI特征及相关因素。方法回顾性分析24例经病理确诊的全瘤型PA病人的临床资料,评估术前病变MRI信号、液液平面、囊肿分隔、蝶窦分型,结合术中所见和组织病理进行分析。结果 24例全瘤型PA病变平均大小(25.41±7.13)mm;蝶窦分型呈全鞍型20例,半鞍型3例,鞍前型1例;MRI上伴液平者16例,无液平者8例;不同性别组在肿瘤大小、囊肿分隔、免疫组化分型间差异具有统计学意义(P0.05)。结论全瘤型PA临床少见,女性相对多发;MRI上伴有液液平面、全鞍型蝶窦以及囊肿分隔表现,有助于临床诊断与鉴别。     

脑分水岭梗死的临床特点及其发病机制研究

目的探讨脑分水岭梗死的临床特点及其发病机制。方法根据脑血管分布的影像学模板,回顾分析124例脑分水岭梗死患者的临床资料,确定并分析皮质分水岭梗死、皮质下型分水岭梗死和混合分水岭梗死3组患者的临床特征及病理生理机制。结果3组脑分水岭梗死患者的临床特征间差别无显著性意义(P＞0．05);皮质分水岭梗死的发病率与皮质下型分水岭梗死间差别有显著性意义(P＜0．001)。其中接受DSA检查的95例患者中,75．0%的皮质分水岭梗死有严重血管狭窄,60．7%皮质下型分水岭梗死有严重血管狭窄,9例混合分水岭梗死患者均有严重血管狭窄。结论分水岭梗死与脑动脉血管狭窄有密切的关系。     

Vascular dementia]

The vast majority of senile dementia consists of Alzheimer's disease and vascular dementia. However, there appear to be geographic differences in the world in the ratio of these two disorders and the types of vascular dementia prevalent in the regions: multi-infarct dementia, lacunar infarct dementia or Binswanger disease. Partly because of those factors, there have been considerable confusions in the classification and diagnostic criteria of vascular dementia. In order to clarify the confusions, it is necessary to recognize the presence of both dementias caused by multiple infarcts as the results of atherothrombosis and embolism, and those caused by multiple lacunar infarcts and periventricular white matter ischemic lesions. Since the clinical manifestations of different types of vascular dementia may be different, it is also necessary to establish the diagnostic criteria which is applicable to all different types of vascular dementia. In order to comprehend the clinicopathologic characteristics of vascular dementia, it is always necessary to analyze the location of arterial occlusion and the mechanism leading to the arterial occlusion.     

青年烟雾病患者的卒中类型及危险因素分析

目的 探索青年烟雾病患者的卒中类型及临床特征,分析青年烟雾病患者发生卒中的危险因素。 方法 回顾性纳入2020年1月-2021年12月解放军总医院第五医学中心收治的青年（18～45岁）卒中型烟雾病患者,将患者分为出血性卒中组和缺血性卒中组进行亚型分析,对比不同卒中类型患者的临床及影像学特征。并以同期未发生卒中的烟雾病患者作为对照组,应用多因素logistic回归分析青年烟雾病患者发生出血性或缺血性卒中的危险因素。 结果 共入组108例卒中型烟雾病患者,其中出血性卒中22例（20.4%）,缺血性卒中86例（79.6%）。出血性卒中组中脑室出血12例（54.5%）,脑实质出血7例（31.8%）,蛛网膜下腔出血3例（13.6%）。缺血性卒中组中大动脉梗死型21例（24.4%）,血流动力学梗死36例（41.9%）,穿支动脉梗死29例（33.7%）。出血性卒中组与缺血性卒中组性别和合并动脉瘤者比例的差异有统计学意义。无卒中对照组共104例,多因素logistic回归分析结果显示,合并动脉瘤（OR?10.569,95%CI?1.524～73.274,P=0.017）为青年烟雾病患者发生出血性卒中的独立危险因素;增龄（OR?1.058,95%CI?1.004～1.115,P=0.034）、合并糖尿病（OR?4.005,95%CI?1.766～9.080,P=0.001）、高铃木分期（OR?1.363,95%CI 1.037～1.793,P=0.027）为青年烟雾病患者发生缺血性卒中的独立危险因素。 结论 青年烟雾病患者的卒中类型以缺血性卒中为主。血流动力学梗死和脑室出血分别是缺血性卒中和出血性卒中的主要类型。增龄、高铃木分期、合并糖尿病和颅内动脉瘤是引起青年烟雾病患者卒中的独立危险因素。     

An update on the diagnosis and treatment of adult Moyamoya disease taking into consideration controversial issues

Abstract

Moyamoya disease (MMD) is characterized by a chronic progressive steno-occlusive disease at the distal portion of the internal carotid artery (ICA) with abnormal Moyamoya vessel (MMV) development without associated diseases. Recent advances in radiologic tests have increased the number of MMD patients. Beyond detection improvement by magnetic resonance angiography (MRA) or cerebral angiography, predicting factors for disease severity, future hemorrhage, clinical outcome, post-op complications, and technical advances in assessing cerebral hemodynamics have been increasingly reported. Although treatment of pediatric MMD is well established, controversy remains over the treatment in adult patients. In particular, there are debates over the disease entity of adult MMD, contralateral progression in adult unilateral MMD, treatment strategy for asymptomatic adult MMD, and the association of MMD with thyroid disease. The purpose of this review is to provide an update on the diagnosis and treatment of adult MMD while addressing controversial issues.     

Primary central nervous system vasculitis and moyamoya disease: similarities and differences

Primary central nervous system vasculitis (PCNSV) and moyamoya disease (MMD) represent rare and poorly-understood causes of stroke. Both may present with similar clinical and auxiliary findings, but differentiation is extremely important because they require different treatment regimens. Our cohort included 21 white patients with PCNSV and 21 white patients with MMD. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. Data were compared between patients with PCNSV and MMD using Chi square test or Fisher’s exact test for categorical data and Mann–Whitney U test for continuous data. The mean age at symptom onset was 42.48 years in PCNSV and 31.0 years in MMD (p = 0.008). All patients with MMD presented with ischemic events while cerebral ischemia was observed in only 14 of 21 patients (66.7%) with PCNSV (p = 0.004). There was no significant difference regarding the frequency of headaches, which represented an important symptom in both conditions. Conventional cerebral angiography verified correct diagnosis in 13 of 17 patients (76.5%) with PCNSV while angiogram verified correct diagnosis in all patients with MMD (p = 0.032). MRI and cerebrospinal fluid studies were appropriate to differentiate between the inflammatory and the non-inflammatory disease. Three PCNSV patients and two MMD patients died within documented follow-up. Despite important pathophysiological and angiographic differences, PCNSV and MMD may present with similar clinical and auxiliary findings. An intensive workup including MRI, conventional cerebral angiography and CSF studies is required to avoid misdiagnosis.     

78例儿童烟雾病和烟雾综合征的临床特征及影像分析

目的 分析儿童烟雾病及烟雾综合征临床特点及影像资料,根据年龄发育阶段研究首发症状及临床 特征,探讨脑梗死发生与颈内动脉病变的关系。 方法 对2002年1月～2009年3月连续住院的78例儿童烟雾病及烟雾综合征患者的病历资料进行回顾 性分析。分析患儿的年龄及性别分布特点,首发症状在不同年龄段、不同类型（出血型和缺血型）中的 特点。将脑血管造影（di gi tal subtracti on angi ography,DSA）图像清晰的缺血型烟雾病分为短暂性脑缺血 发作（transient ischemic attack,TIA）组和脑梗死（cerebral infarction,CI）组,比较两组的Suzuki分期差异。 结果 78例患儿中男36例（46.2%）,女42例（53.8%）。起病年龄18个月～17岁,平均（8.55±3.80）岁, 5～10岁患儿47例（60.3%）。幼儿期首发症状仅见肢体无力和抽搐,发作性头痛从学龄前期开始出 现,学龄期开始出现视力障碍和不自主运动,记忆力下降等。脑缺血型烟雾病72例（92.3%）。缺血 型首发症状多见TIA 31例（43.1%）、肢体瘫痪15例（20.8%）、发作性头痛22例（30.6%）、肢体麻木11 例（15.3%）、抽搐8例（11.1%）、言语障碍6例（8.3%）等。出血型首发症状为头痛伴意识障碍（50%）、 伴肢体无力（50%）、伴言语障碍（16.7%）。CI部位多在额颞顶叶,脑血管病变主要累及颈内动脉系统。 出血部位多在脑室系统（50%）,其次为基底节（33.4%）,丘脑和额颞叶各占16.7%。TI A组（27例）和CI 组（27例）脑血管造影Suzuki分期无显著性差异（χ2=1.034,P =0.596）。 结论 5～10岁为儿童烟雾病的发病高峰期,临床以缺血型为主,出血型少见。不同年龄发育阶段临 床首发症状有所差异。以TIA和CI为表现的缺血型烟雾病患者DSA的Suzuki分期无显著差异。     

既往卒中病史对缺血性脑血管病患者卒中结局的影响研究

目的 探讨中国人群中首发和复发缺血性脑血管病患者的临床特征和卒中结局差异。 方法 本研究基于全国多中心前瞻性中国国家卒中登记研究Ⅲ（the third China national stroke regi stry,CNSR-Ⅲ）,连续纳入2015年8月-2018年3月急性缺血性卒中或TIA患者,收集人口学信息、血 管危险因素、既往用药史及病因分型系统（causative classification system,CCS）等临床资料,记录随 访3个月和1年时卒中结局。卒中结局包括卒中复发（缺血性卒中或出血性卒中）、联合血管事件（卒中、 心肌梗死及血管性死亡事件）、脑血管病源性死亡及不良功能结局（mRS＞2分）。依据患者既往是否 有卒中病史分为有卒中病史组和无卒中病史组,比较两组的临床特征及卒中结局差异,并分析卒中病 史与卒中结局间的关系。 结果 最终纳入15 166例患者,平均年龄62.2±11.3岁,其中女性4802例（31.7%）;有卒中病史患者 3355例,无卒中病史患者11 811例。有卒中病史组患者年龄,冠心病、高血压、脂代谢紊乱、糖尿病、心 房颤动比例,既往用药史比例、入院NIHSS评分、住院期间降糖和降压治疗比例均高于无卒中病史组, 目前吸烟和重度饮酒比例、入院时LDL-C水平及住院期间抗血小板治疗比例低于无卒中病史组,差 异均有统计学意义。两组CCS分型的分布差异有统计学意义,其中有卒中病史组大动脉粥样硬化型和 心源性栓塞型卒中比例高于无卒中病史组。多因素分析结果显示,卒中病史是随访3个月不良功能结 局（校正OR 1.25,95%CI 1.09～1.44,P =0.002）,随访1年卒中复发（校正HR 1.44,95%CI 1.25～1.67, P＜0.001）、联合血管事件（校正HR 1.43,95%CI 1.24～1.64,P＜0.001）、脑血管病源性死亡（校正 HR 1.42,95%CI 1.12～1.80,P =0.004）、不良功能结局（校正OR 1.63,95%CI 1.42～1.88,P＜0.001）的 危险因素。 结论 有无卒中病史的缺血性卒中患者的临床特征及随访结局差异较大,尽管患者进行卒中二级 预防治疗,卒中病史仍然是患者1年卒中复发、联合血管事件、脑血管病源性死亡及不良功能结局的 危险因素。     

78例儿童烟雾病和烟雾综合征的临床特征及影像分析

目的分析儿童烟雾病及烟雾综合征临床特点及影像资料,根据年龄发育阶段研究首发症状及临床特征,探讨脑梗死发生与颈内动脉病变的关系。方法对2002年1月~2009年3月连续住院的78例儿童烟雾病及烟雾综合征患者的病历资料进行回顾性分析。分析患儿的年龄及性别分布特点,首发症状在不同年龄段、不同类型(出血型和缺血型)中的特点。将脑血管造影(digital subtraction angiography,DSA)图像清晰的缺血型烟雾病分为短暂性脑缺血发作(transient ischemic attack,TIA)组和脑梗死(cerebral infarction,CI)组,比较两组的Suzuki分期差异。结果 78例患儿中男36例(46.2%),女42例(53.8%)。起病年龄18个月~17岁,平均(8.55±3.80)岁,5~10岁患儿47例(60.3%)。幼儿期首发症状仅见肢体无力和抽搐,发作性头痛从学龄前期开始出现,学龄期开始出现视力障碍和不自主运动,记忆力下降等。脑缺血型烟雾病72例(92.3%)。缺血型首发症状多见TIA 31例(43.1%)、肢体瘫痪15例(20.8%)、发作性头痛22例(30.6%)、肢体麻木11例(15.3%)、抽搐8例(11.1%)、言语障碍6例(8.3%)等。出血型首发症状为头痛伴意识障碍(50%)、伴肢体无力(50%)、伴言语障碍(16.7%)。CI部位多在额颞顶叶,脑血管病变主要累及颈内动脉系统。出血部位多在脑室系统(50%),其次为基底节(33.4%),丘脑和额颞叶各占16.7%。TIA组(27例)和CI组(27例)脑血管造影Suzuki分期无显著性差异(χ2=1.034,P=0.596)。结论 5~10岁为儿童烟雾病的发病高峰期,临床以缺血型为主,出血型少见。不同年龄发育阶段临床首发症状有所差异。以TIA和CI为表现的缺血型烟雾病患者DSA的Suzuki分期无显著差异。     

Familial occurrence of moyamoya disease: a clinical study

Background We reviewed a consecutive series of moyamoya disease (MMD) in children and studied their familial pedigrees to determine whether they showed specific clinical features or patterns of inheritance, and to investigate any correlation between familial MMD and common Asian diseases.Methods Cases of familial MMD (N=10) were reviewed in the aspect of clinical presentation, such as, symptoms and signs, age of onset, imaging studies including magnetic resonance imaging (MRI), cerebral angiography, and single photon emission computed tomography (SPECT), and operative results including complications, to identify differences between these patients and those with sporadic MMD (N=194). The male to female ratio in those with familial MMD was 4:6 and mean age was 8 years (3–17). All were ischemic cases and five showed cerebral infarction on MRI. As a preliminary genetic study, familial pedigrees were examined. In addition, their familial histories concerning common Asian diseases, such as, hepatic disease, cancers, stroke, coronary heart disease, amyloidosis, and systemic lupus erythematosus, were investigated by telephone survey.Results and conclusions The familial MMD cases did not reveal any differences from the other MMD children in terms of clinical findings, imaging data, or surgical results. In our series, five cases (50%) showed MMD between siblings. Familial MMD relations were also observed with cousins, a mother, and an aunt. No specific pattern of genetic inheritance was observed, and no relation was found between the familial occurrence of MMD and common Asian diseases.     

缺血型和出血型成年烟雾病患者MMP与VEGF的表达水平差异及其与病情程度的关系

目的 探讨缺血型和出血型成年烟雾病患者MMP与VEGF的表达水平差异及其与病情程度的关系。方法 选取 2014年1月-2017年1月于本院治疗的80例烟雾病患者,其中出血型34例,缺血型46例,同时选取同期在本院进行体检健康的60例患者,比较3组患者的血清MMP-9及VEGF水平; 比较出血型和缺血型烟雾病患者Suzuki分级中血清VEGF和MMP-9水平; 根据病情进程,将患者分为好转组和非好转组,比较2组患者发病后第3 d及第15 d的血清VEGF和MMP-9水平。结果 出血型和缺血型烟雾病患者的血清VEGF和MMP-9水平均高于对照组(P<0.05),但两者之间的VEGF和MMP-9水平无明显差异(P>0.05); 随着Suzuki分级的增加,2组患者的VEGF和MMP-9水平也相应增加,且有明显差异(P<0.05); 好转组患者入院后第3 d和第15 d的血清VEGF和MMP-9水平均低于非好转组(P<0.05)。结论 血清MMP与VEGF水平在缺血型和出血型成年烟雾病患者中呈高表达,且与病情严重程度有关。     

Coexistence of moyamoya and Graves’ diseases: The clinical characteristics and treatment effects of 21 Chinese patients

Objective

To investigate the clinical features, pathogenesis and treatment effect of patients who had moyamoya disease (MMD) concurrent with Graves’ disease (GD).

Patients and methods

Retrospective analysis of the examination and treatment results of the MMD patients with GD from January 2003 to April 2012 treated by our teamwork.

Results

There were 1493 MMD patients included in this retrospective study. Among them, there were 16 female and 5 male MMD patients coexisted with Graves’ diseases, and 15 of them were adult patients. Bilateral and unilateral lesions were observed in 18 and 3 cases, respectively. 19 patients show cerebral infarction. 18 patients manifested MMD symptoms when they were in a hyperthyroid state. 17 patients received encephaloduroarteriosynangiosis (EDAS) surgery after antithyroid treatment. There were 20 patients completed 2 and 77 months follow-up. 17 patients had good recovery and the other 3 patients had mild or moderate disability. Post-operative DSA achieved in 8 patients examinations showed good collateral circulation to the brain from superficial temporal artery (STA).

Conclusion

Moyamoya disease complicating GD is mostly occurred in adult female patients. Their clinical symptoms are mainly ischemic lesions and usually occur during the state of hyperthyroidism. The pathogenesis of moyamoya disease complicating GD may be associated with a variety of genetic and immune factors. Surgical treatment can establish effective collateral circulation and reduce the risk of recurrence of stroke.