烟雾病合并Graves病1例报告

烟雾病(Moyamoya disease)是以双侧颈内动脉远端严重狭窄或闭塞,脑底及脑表面烟雾状小血管形成为特征的慢性脑血管闭塞性疾病,是引起卒中的少见原因.Graves病是以体内过多甲状腺激素形成为特点的一种自身免疫性疾病,过多的甲状腺激素会改变血管的反应性进而损害动脉壁[1].烟雾病合并Graves病国内较少报道,现将本院收治的1例烟雾病合并Graves病患者的临床特征及诊疗情况报道如下.     

出血型烟雾病的临床特征

烟雾病是一种慢性进展性脑血管病,临床上可以脑缺血或脑出血发病。出血型烟雾病合并 动脉瘤是影响预后的重要因素,脉络膜前动脉的破裂出血是引起烟雾病脑出血的主要原因,采取积极 有效的措施避免再出血的发生是治疗的关键。     

烟雾病合并甲状腺功能亢进症10例临床分析

目的探讨烟雾病合并甲状腺功能亢进症(甲亢)的临床特点及其可能的发病机制。方法回顾性分析10例烟雾病合并甲亢患者的临床资料。结果本组10例患者中,男1例,女9例。所有患者均表现为缺血性脑血管病。其中DSA确诊烟雾病1例,CTA确诊4例,MRA确诊5例;双侧颈内动脉系统受累9例,单侧受累1例。结论对于烟雾病患者,尤其是女性,应常规行甲状腺功能筛查;而甲亢患者也应行脑血管病变筛查,以尽早发现脑血管病变,早期预防,避免卒中。     

烟雾病合并甲状腺功能亢进:两例报道

正烟雾病合并甲状腺功能亢进是烟雾病中的一种特殊类型,文献报道较少且病因不明。2011年3月至2012年12月济宁医学院附属医院收治了两例烟雾病合并甲状腺功能亢进患者,现报道如下。烟雾病的诊断标准符合日本厚生省烟雾病诊断标准(1996),甲状腺功能亢进的诊断标准符合内科学(人民卫生出版社,第七版)中Graves病的诊断标准。病例1:女性,20岁,因"一过性肢体无力伴意识丧失多次,频发1月余"于2011年3月17日收入院,患者平素生活规     

27例成人烟雾病的临床特点分析

目的探讨成人烟雾病患者的临床特点,以提高对烟雾病的认识。方法回顾徐州医学院附属医院神经内科2010年1月～2011年12月收治的27例初次诊断烟雾病住院患者的临床资料,分析烟雾病的发病年龄、性别和伴发疾病的特点。结果本组患者发病平均年龄39．2岁,其中男性患者为33．6岁,女性为41．5岁,不同性别患者组发病年龄比较差异无显著性;不同性别患者间临床表现分布无显著性差异;男女患者比例1：2．38,发病频数分布拟合优度检验差异具有显著性（x2=4．481,P=0．034）;患者起病形式为缺血性卒中表现者占59．25％;本组中3例累及椎基底动脉系统,占11．11％;40．74％患者合并高血压。结论本组成人烟雾病患者中女性患者比例高于男性,多数患者临床表现为缺血性脑卒中。该病受累血管可以合并椎一基底动脉系统。     

儿童烟雾病与成人烟雾病79例临床及影像学分析

目的 分析并总结儿童烟雾病与成人烟雾病的临床及影像表现的差异.方法 对回顾性分析79例烟雾病患者的临床资料,儿童30例,成人49例,对其临床症状和影像特征进行对照研究.结果 儿童组首次发病以缺血性脑血管病表现为主,显著高于成人组（P＜0.05）,成人组首次发病以出血性脑血管病表现为主,显著高于儿童组（P＜0.05）.结论 儿童烟雾病与成人烟雾病的临床症状和影像表现具有差异,临床接诊应区别对待.     

国人烟雾病临床特征探讨

目的探讨中国烟雾病人的临床特征。方法将54例中国人烟雾病的发病年龄、性别、临床症状、影像及治疗的特点，与日韩及欧美烟雾病患者进行了分析对比：结果中国人烟雾病的发病年龄似有日韩一样的两个高峰，但成人及男性患者发生率高于日韩，脑梗塞发生率也较日本高，脑短暂缺血性发作及癫痫的发生率却低于日本，脑出血的发生率与日本相近且以女性患者多见，两国患者的治疗效果无明显差异：结论中国人烟雾病的临床特征与日韩欧美患者不完全相同，其发病机理可能存在一些差异。     

烟雾病免疫机制研究进展

烟雾病(Moyamoya disease)又称自发性Willis环闭塞症,是一种慢性进行性闭塞性脑血管疾病,以双侧颈内动脉末端及大脑前动脉、大脑中动脉起始部动脉内膜缓慢增厚,动脉管腔逐渐狭窄以至闭塞,脑底穿通动脉代偿性扩张为特征。目前烟雾病的病因尚不明确。本文从免疫学方面对烟雾病的发病机制进行综述,旨在更深入的了解烟雾病,期望能够对临床有一定指导意义。     

烟雾病的临床特点分析

目的:观察烟雾病的临床特点,为临床治疗缺血性脑血管病提供依据.方法:回顾性分析20例烟雾病患者的临床资料,观察其临床特点.结果:本组患者年龄在46～60岁,平均(51.25±4.40)岁,临床表现多样,头颅影像学有改变;脑血管数字减影血管造影(DSA)可见明显烟雾病表现.结论:烟雾病是一种较少见的慢性脑血管进行性闭塞性疾病,是缺血性脑血管病发病的重要原因之一,应尽早行脑血管DSA检查,及时治疗.     

出血型烟雾病的临床特征及治疗

目的 分析出血型烟雾病病例的临床特征,探讨烟雾病出血的病因及治疗方法.方法 回顾性分析61例出血型烟雾病发病特征、出血部位及类型、脑血管造影特点及治疗结果.结果 61例病例中成人57例,以脑出血破入脑室发病居多.52个出血半球存在脉络膜前动脉异常扩张合并后交通动脉扩张.61例患者共行硬脑膜颞浅动脉血管融通术116例次,行硬脑膜枕动脉血管融通术2例次,2例未行手术治疗,后因再发脑出血死亡,手术后无再发出血病例.结论 脉络膜前动脉合并后交通动脉扩张是造成烟雾病出血的重要原因之一;行脑硬膜动脉血管融通术可能有益于预防再出血的发生.     

Disappearance of aneurysms associated with moyamoya disease after STA-MCA anastomosis with encephaloduro myosynangiosis

Moyamoya disease is a rare cerebrovascular disease characterized by steno-occlusive vasculopathy affecting the terminal internal carotid arteries. Although the effect of direct arterial bypass on the prevention of recurrent haemorrhage or ischemic events in patients with hemorrhagic moyamoya disease has been demonstrated, disappearance of aneurysms associated with moyamoya disease has rarely been reported. In this study, we present two patients with aneurysms associated with moyamoya disease. After superficial temporal artery to middle cerebral artery anastomosis combined with encephaloduro myosynangiosis, the aneurysms on the moyamoya vessels disappeared, which was confirmed by follow-up angiography.     

原发性脑室出血型Moyamoya病21例临床分析

目的通过回顾性分析我院10年来收治的21例原发性脑室出血型Moyamoya病患者的临床资料,探讨非高血压性原发性脑室出血与Moyamoya病及其影像学特征的关系。方法对我院神经外科10年来确诊的原发性脑室出血Moyamoya病患者的CT、DSA和临床治疗进行回顾性分析。结果 25例非高血压性原发性脑室出血患者中DSA证实21例为Moyamoya病。3例合并基底动脉动脉瘤。所有患者出血侧均存在脉络膜前动脉异常扩张扭曲及异常分支。该组患者首次治疗均获得良好结果。结论 Moyamoya病是成人非高血压性原发性脑室出血的主要病因,对成人非高血压性原发性脑室出血应常规行DSA明确是否存在Moyamoya病。成人非高血压性原发性脑室出血与脉络膜前动脉的扩张扭曲或异常分支密切相关,并且后者可作为前者的预测指标。     

Cystic neurohypophysial germinoma associated with moyamoya disease

Moyamoya disease associated with a germinoma has never yet been reported in the literature. This report describes a case of a cystic neurohypophysial germinoma associated with moyamoya disease. A 15-year-old girl with central diabetes insipidus was admitted to the hospital. Radiological examination revealed a suprasellar tumor and moyamoya disease. Surgery and pathological study confirmed pure germinoma. After the patient was treated with radiation and chemotherapy, the tumor was controlled without any effect on the moyamoya vessels. This report describes a rare incidental combination of moyamoya disease and a suprasellar germinoma. In the management of such cases, physicians should be aware of the symptoms, course, and natural history of both diseases and the effects and side effects of each type of therapy.     

Moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma

Moyamoya disease has been associated with renal artery stenosis, cerebral hemorrhage, and multiple cranial traumas. We report a unique case of moyamoya disease associated with polycystic kidney disease and eosinophilic granuloma. Although the etiology of moyamoya disease is unknown, a familial pattern of occurrence has been documented. Of particular importance is its presentation with polycystic kidney disease, an autosomal dominant disease, suggesting a hereditary component to the etiology of this unusual vasculitic disease.     

Moyamoya disease associated with hemoglobin Fairfax and beta-thalassemia

Moyamoya disease is a rare cerebrovascular condition characterized by steno-occlusive disease of the major intracranial arteries at the base of the brain, and the appearance of innumerable, tiny, arterial collaterals that resemble a "puff of smoke." Although it has been associated with hematologic diseases such as sickle-cell disease, the association with other hemoglobinopathies is less frequently observed. We describe the association of a unique hemoglobinopathy (hemoglobin Fairfax) with beta-thalassemia and moyamoya disease in a 9-year-old girl with a history of stroke. To our knowledge, this is the first report of this unstable hemoglobin with moyamoya disease, and it emphasizes the potential for cerebral infarction due to the severe anemia of hemolytic disease.     

动脉粥样硬化性大脑中动脉狭窄继发Moyamoya 综合征的脑血管病变及侧支循环分布模式

目的：探讨动脉粥样硬化性大脑中动脉狭窄继发 moyamoya 综合征的脑血管病变及侧支循环分布模式。方法收集2010年8月~2013年12月经 DSA 证实动脉粥样硬化性大脑中动脉狭窄患者26例。评估 moyamoya 血管网建立与颅内血管病变及软脑膜侧支循环开放度之间的关系。结果26例患者中 moy-amoya 综合征组17例,非 moyamoya 综合征组9例。moyamoya 综合征组4例患者在 MCA 闭塞侧 A1段表现为细小或缺如,而非 moyamoya 综合征组9例 A1段表现正常。moyamoya 综合征组11例在 MCA 一侧或对侧的 ACA 上发现动脉粥样硬化性狭窄或闭塞病变,而非 moyamoya 综合征组未发现上述病变（P =0.004）。非 moyamoya 综合征组所有患者均显示自 ACA 到达 M1或 M2节段足够的软脑膜侧支循环,而 moy-amoya 综合征组仅有8例（P =0.012）。结论动脉粥样硬化所致的 moyamoya 血管网重建可能与软脑膜侧支循环建立不佳有关。     

Inheritance of moyamoya disease in a Caucasian family

Background and purpose: Moyamoya disease is a very rare occlusive cerebrovascular disorder characterized by progressive stenosis or occlusion of the intracranial portion of the internal carotid artery and proximal cerebral arteries with an extensive network of fine collaterals. The aetiology and genetic susceptibility of moyamoya disease, especially in Caucasians, still remains unclear. Methods and results: We describe the cases of affected German father, daughter and son with juvenile stroke because of idiopathic moyamoya disease. The rare existing literature is reviewed and discussed. Conclusions: This is the first report on a father‐to‐child inheritance pattern in Caucasian patients with idiopathic Moyamoya disease (MMD). Our cases indicate possible genetic risk factors for the genesis of Caucasian Moyamoya disease.     

A case of unilateral moyamoya disease presenting with hemichorea

We reported a 12-year-old boy with unilateral moyamoya disease whose initial and predominant manifestation was hemichorea. Neurological examinations revealed chorea in his left upper extremity and muscle hypotonia in his left upper and lower extremities. Cranial MRI showed moyamoya vessels only in the right basal ganglia and infarction in the white matter of the right frontal lobe. Right carotid angiography revealed stenosis in the distal part of internal carotid artery, and in the proximal part of anterior and middle cerebral arteries with moyamoya vessels. Left carotid angiography showed normal findings. He was diagnosed as a suspected case of moyamoya disease (unilateral moyamoya disease) according to the diagnostic criteria proposed by the Research Committee on Moyamoya Disease of the Ministry of Health and Welfare of Japan. His chorea responded to haloperidol but encephalo-duro-arterio-synangiosis on the right side improved all symptoms. Chorea occurs in some patients with moyamoya disease. Hypofunction of the striatal indirect pathway is suggested as the cause of chorea. In this case an ischemic lesion in the right striatum may have caused hypofunction of the pathway and developed chorea and hypotonia.     

A case of moyamoya disease presenting with chorea

Case report We describe a 15-year-old girl with moyamoya disease whose initial manifestation was chorea-like involuntary movements. T2-weighted magnetic resonance imaging showed high signal intensity lesions in the left frontal lobe, right parieto-occipital lobes, and frontal subcortical white matter. Single-photon emission computed tomography (SPECT) showed diffuse hypoperfusion of the whole brain. Bilateral direct and indirect cerebrovascular bypass surgeries were performed. Chorea disappeared 2 days after the surgery. Follow-up SPECT demonstrated increased cerebral perfusion in the bilateral frontal, temporal, and parietal regions.Conclusions Chorea accompanied with moyamoya disease can be properly managed by revascularization surgery. Moyamoya disease should be remembered as being one of the differential diagnoses of chorea, which is treatable by surgery.     

Cerebral infarction due to moyamoya disease in young adults

Moyamoya disease was diagnosed as the cause of cerebral infarction in eight young adults (seven women, one man), aged 17-40 (mean 33) years. All had angiographic abnormalities characteristic of moyamoya disease. Single-photon emission tomography showed bilateral carotid circulation hypoperfusion and posterior circulation hyperemia in all seven patients with regional cerebral blood flow studies. All seven women had used oral contraceptives before cerebral infarction. Four patients were treated medically; one died of a second cerebral infarction 9 months after diagnosis. Four patients underwent superficial temporal-to-middle cerebral artery anastomosis; they did well. Moyamoya disease should be included in the differential diagnosis of cerebral infarction as well as intracranial hemorrhage in young adults, particularly women. A possible relation between moyamoya disease and oral contraceptive use deserves investigation.