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首页> 外文期刊>Journal of stroke and cerebrovascular diseases: The official journal of National Stroke Association >Moyamoya Disease Associated with Graves' Disease and Down Syndrome: A Case Report and Literature Review
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Moyamoya Disease Associated with Graves' Disease and Down Syndrome: A Case Report and Literature Review

机译：与坟墓疾病和唐氏综合征相关的Moyamoya病：案例报告和文献综述

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Background: Moyamoya vessels are cerebral vasculopathies characterized by net-like collateral vessel formation at the cerebral basal area and stenosis of the terminal internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery. A diagnosis of Moyamoya disease depends on the bilateral presence of Moyamoya vessels. Moyamoya disease associated with Graves' disease has rarely been reported to be a cause of ischemic events due to hyperthyroidism. However, there are extremely rare cases of Moyamoya disease with concurrent Graves' disease and Down syndrome. We aimed to report such a case, and to compare these cases' clinical features, pathogenesis, and treatment effects to those of the cases of concurrent Moyamoya disease and Graves' disease alone. Methods: We performed an English literature search using the PubMed database and the keywords Moyamoya, quasi-Moyamoya, Graves' disease, thyrotoxicosis, Down syndrome, and trisomy 21. Results: Only five cases of Moyamoya disease with Graves' disease and Down syndrome have been reported, including our own. Four patients were female (80%), and all underwent antithyroid therapy and experienced ischemic episodes, including transient ischemic attacks. At the time of their vascular accident, two patients were in a thyrotoxic state; only our patient was in a euthyroid state. The mean age was 15.6 years (standard deviation: 6.1), which was younger than the mean age of 31.4 years (standard deviation: 13) in patients with Moyamoya disease and Graves' disease alone. Down syndrome is commonly associated with abnormal vascular networks due to increased endostatin concentrations or immunological abnormalities such as those that occur in Graves' disease. Graves' disease accelerates the progression of Moyamoya disease and ischemic attacks due to atherosclerosis, enhances sympathetic nervous system activity and immunological changes. As compared to Moyamoya disease patients, patients with concurrent Graves' disease only and Moyamoya disease patients with concurrent Graves' disease and Down syndrome may experience accelerated disease progression or more frequent ischemic attacks. Conclusion: Early imaging follow-ups and strict control of thyroid function are necessary in such cases; if ischemic attacks have already occurred, revascularization surgery may be effective.
机译：背景：烟雾血管是一种脑血管病，其特征是大脑基底区形成网状侧支血管，颈内动脉末端、大脑中动脉近端和大脑前动脉狭窄。烟雾病的诊断取决于双侧烟雾血管的存在。与格雷夫斯病相关的烟雾病很少被报道为甲亢引起的缺血性事件的原因。然而，伴有格雷夫斯病和唐氏综合征的烟雾病病例极为罕见。我们的目的是报告这样一个病例，并将这些病例的临床特征、发病机制和治疗效果与合并烟雾病和Graves病的病例进行比较。方法：我们使用PubMed数据库和关键词Moyamoya、准Moyamoya、Graves病、甲状腺毒症、唐氏综合征和21三体进行英文文献检索。结果：仅报告了5例伴有Graves病和唐氏综合征的烟雾病病例，包括我们自己的病例。四名患者为女性（80%），均接受了抗甲状腺治疗，并经历了缺血发作，包括短暂性脑缺血发作。在血管事故发生时，两名患者处于甲状腺毒性状态；只有我们的病人处于甲状腺功能正常状态。平均年龄为15.6岁（标准差：6.1），比单纯患有烟雾病和格雷夫斯病的患者的平均年龄31.4岁（标准差：13）年轻。唐氏综合征通常与内皮抑素浓度升高或格雷夫斯病等免疫异常引起的异常血管网络有关。格雷夫斯病加速了烟雾病和动脉粥样硬化引起的缺血性发作的进展，增强了交感神经系统的活动和免疫变化。与烟雾病患者相比，仅合并Graves病的患者和合并Graves病和唐氏综合征的烟雾病患者可能会经历疾病进展加快或更频繁的缺血性发作。结论：早期影像学随访和严格控制甲状腺功能是必要的；如果缺血发作已经发生，血管重建手术可能是有效的。

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