MPO-ANCA阳性的多脑神经炎(附1例报告及文献复习)

目的报道1例以多脑神经炎为主要临床表现的抗中性粒细胞胞质抗体(ANCA)中的抗髓过氧化物酶蛋白(MPO)阳性患者,并结合文献复习对本病进行回顾性分析。方法以"ANCA、MPO,血管炎,脑神经"等关键词在维普、中国知网等数据库进行检索,在Pub Med数据库以"ANCA,MPO,vasculitis,cranial neuropathies"等关键词进行检索,获取文献报道35例累及脑神经的MPO阳性ANCA相关血管炎(AAV)患者,分析本病例以及文献报道病例的临床特点。结果本研究报道国内首例以不伴有肥厚性硬脑膜炎的多脑神经炎为主要临床表现的MPO-ANCA阳性患者。结合文献报道共36例累及脑神经的MPO-AAV患者,年龄46～80岁,男女性别比为1∶1,以日本和中国病例居多;多脑神经较单脑神经受累更常见,受累脑神经中以听神经最为常见,其次为面神经、外展神经及三叉神经;合并症状:以肥厚性硬脑膜炎最多见,其次为鼻窦炎、中耳炎等耳鼻喉科症状。结论 MPO-AAV临床表现广泛且涉及各器官,部分可出现脑神经损害,依据抗体对AAV患者进行分类较临床征候学分型更具可操作性。     

以神经系统损害为首发症状的抗中性粒细胞胞质抗体相关性血管炎1例报道

<正>抗中性粒细胞胞质抗体(Anti-neutrophil cytoplasmic antibodies,ANCA)相关性血管炎(ANCA-associated systemic vasculitis,AASV)是一组由不同病因引起的以血管壁炎症和纤维素样坏死为病理特征的系统性疾病。神经系统损害是AASV的常见症状之一,部分患者为首发症状,但该病比较少见,在临床中较易被忽视[1]。现报道我科诊治1例以神经系     

继发于抗中性粒细胞胞浆抗体相关系统性血管炎的肥厚性硬脊膜炎1例报告

正肥厚性硬脊膜炎(HSP)是一种以硬脊膜增厚和炎症性纤维化为特征的罕见疾病~([1-2])。抗中性粒细胞胞浆抗体(ANCA)与HSP的关系目前关注较多,但多数只累及颅内硬脑膜。现报告1例继发于ANCA相关系统性血管炎(AASV),并仅累及胸段脊膜的HSP,以期提高对本病的认识。1病例女,75岁,因"后背疼痛、行走困难5个月,加重10d"于2016年10月20日入院。2016年5月患者无诱因出现     

脑淀粉样血管病1例尸检报道

目的 探讨脑淀粉样血管病相关脑出血的临床表现和病理特点。方法 回顾性分析1例脑淀粉样血管病患者的临床表现，通过尸检观察病理改变。结果 脑淀粉样血管病相关脑出血临床上呈复发性和多灶性，刚果红染色可见淀粉样物质沉积于血管壁。结论 对于临床上出现的复发性和多灶性的脑出血，尤其是脑叶出血，要警惕脑血管淀粉样变性的可能，脑活检刚果红染色可以协助诊断。     

以脑梗死为首发表现的ANCA相关性小血管炎1例报告

正抗中性粒细胞胞质抗体(antineutrophil cytoplasmic antibody,ANCA)相关性小血管炎(ANCA-associated systemic vasculitis,AASV)是一组可累及全身小血管的自身免疫性疾病,先后出现多系统多器官症状,绝大多数病例是在患者出现具有明确免疫色彩的全身症状时诊断的,该病对神经系统主要累及周围神经,在临床工作中遇到1例以脑梗死为首发临床     

胶原血管病伴肌炎69例临床和病理研究

目的:研究胶原血管病伴肌炎(CTM)的临床、病理特征。方法:回顾性总结69例CTM患者的临床资料,研究其临床表现、血清免疫学、肌酶学、肌电图、肌肉病理的特点。结果:本病临床上主要有肌无力、肌痛、关节痛、发热、肌萎缩、雷诺征等;血清免疫学可见免疫球蛋白增高和补体降低,部分患者ANA、ENA阳性;CK等血清肌酶增高不及单纯性多发性肌炎(PM)明显;肌电图呈肌原性损害,也可有神经原性损害。肌肉病理显示免疫炎性改变,表现为区域性肌纤维变性坏死,肌间质血管狭窄、闭塞、炎症细胞浸润,血管炎和肌束周萎缩远较PM常见(P<0.01)。结论:CTM的临床和肌肉病理与PM有所不同,广泛的血管炎性病变是CTM特征性的病理改变。     

系统性红斑狼疮神经系统病变的临床及病理特征

目的探讨系统性红斑狼疮（SLE）神经系统病变的临床及病理特征。方法回顾性分析6例SLE神经系统病变患者的临床及病理资料。结果本组患者累及中枢神经系统4例,其中癫痫1例、脑梗死2例、白质脑病1例;多发性单神经病3例,腓肠神经活检示1例有典型血管炎改变,2例无血管炎改变,腓肠神经内可见有髓纤维丢失、轴索和髓鞘断裂呈块状深染及髓磷脂小球形成。肌活检1例示肌纤维轻度变性坏死。皮肤活检3例示胶原变性,小静脉周围可见炎性细胞浸润。结论SLE可导致神经系统广泛病变,故其临床表现和病理改变复杂多样。     

ANCA、ACA与脑卒中发病关系的研究

1资料和方法脑卒中患者81例中,男54例、女27例,年龄38~74岁,平均59·9岁;其中脑梗死53例,脑出血28例。所有患者均经头颅CT证实。同时期献血员100名作为对照。抗中性粒细胞胞浆抗体(ANCA)采用间接免疫荧光法测定。抗心磷脂抗体(ACA)采用ELISA法测定。计数资料的组间比较采用卡方检验,显著水平设为0·05。2结果81例脑卒中患者中,血清ANCA阳性41例(50·6%),而对照组为2例(2·0%),两组间比较差异有统计学意义(P<0·01)。脑梗死组ANCA阳性率为50·9%(27/53),脑出血组为50·0%(14/28),两组ANCA阳性率均显著高于对照组(P<0·01),但两组…     

淀粉样变脑出血的临床特点及诊断方法

目的 分析淀粉样变脑出血的临床特点及诊断方法.方法 对55例自发性脑出血患者的临床表现及预后进行总结,对病理标本进行刚果红特殊染色和β淀粉样蛋白免疫组化染色,以明确病理诊断.结果 55例中6例首诊为可疑性淀粉样变脑出血,1例通过病理染色予以排除;2例术前诊断为可疑高血压性脑出血的患者经病理染色确诊为淀粉样变脑出血.结论 淀粉样变脑出血术前可以根据临床特点做出初步诊断;但是对于临床表现不典型者,诊断上还有一定的困难,病理分析仍然是诊断的金标准.目前对于此病的临床诊断方法需进一步研究改进.     

中青年脑梗死与脑出血临床对比分析

目的 探讨中青年脑梗死与脑出血的病因、危险因素、临床特点的异同,为中青年脑血管疾病的防治提供理论依据.方法 回顾性分析中青年脑梗死130例与108例青年脑出血患者临床资料.结果 (1)中青年脑梗死以动脉粥样硬化、高血压、血管炎为主要病因,分别占36.9%、21.5%、20.8%;中青年脑出血以高血压、脑血管畸形、动脉粥样硬化为主要病因,分别占32.4%、24.1%、16.7%.(2)2组病人的病变部位均以基底节区和脑叶最常见,其次为脑干、丘脑.结论 中青年脑梗死与脑出血的病因差异显著;中青年脑梗死与脑出血危险因素相同;中青年脑梗死临床治疗和预后较脑出血好.     

Predictive factors for intracerebral hemorrhage in patients with cavernous angiomas

OBJECTIVES: Prediction of intracerebral hemorrhage (ICH) in patients with cavernous angiomas is not totally elucidated. The aims of our study were to determine the rate of cerebral hemorrhage, its associated factors, and the clinical outcome in patients with cavernous angiomas in a Hispanic population. METHODS: We studied 133 patients with cavernous angiomas. The patients were classified into two groups depending on whether they presented an ICH. A comparative analysis of demographics and clinical data, neuroimaging characteristics, and prognosis was carried out in patients with and without hemorrhage. The hemorrhage rate (expressed as the percentage per patient per year) was also estimated. RESULTS: Seventy-eight patients (59%) had hemorrhage. Non-lobar location of angiomas was associated with hemorrhage [OR 4.82 (CI 95% 2.17-10.73; p=<0.001)]. In contrast, factors associated with a decreased risk of hemorrhage were a family history of epilepsy [OR 0.30 (CI 95% 0.10-0.79; p=0.016)] and lobar location of the angiomas [OR 0.21 (CI 95% 0.09-0.46; p=<0.001)]. The hemorrhagic rate of 1.71% per patient per year was influenced by the location. It was only 1.22% per patient per year in lobar angiomas and 2.33, 2.39, and 2.82% per patient per year for brainstem, cerebellum, and deep hemispheric angiomas, respectively. CONCLUSIONS: The non-lobar location of cavernous angiomas gives a higher risk of hemorrhage in our Mexican mestizo population, without the hemorrhage being related to either age or sex.     

Intraventricular Glioblastoma Multiforme with Previous History of Intracerebral Hemorrhage : A Case Report

GBM is the most common primary brain tumor, but intraventricular GBM is rare and only few cases have been reported in the literature. The authors report a case of 64-year-old man who had a remote history of previous periventricular intracerebral hemorrhage. Brain computed tomography (CT) and magnetic resonance (MR) imaging showed an intraventricular lesion with inhomogeneous enhancement, infiltrative borders and necrotic cyst, and obstructive hydrocephalus. The patient underwent surgical removal through transcortical route via the bottom of previous hemorrhage site and the final pathologic diagnosis was GBM. We present a rare case of an intraventricular GBM with detailed clinical course, radiological findings, and pathological findings, and the possible origin of this lesion is discussed.     

Predictive Factors for Intracerebral Hemorrhage in Patients with Cavernous Angiomas

Abstract

Objectives: Prediction of intracerebral hemorrhage (ICH) in patients with cavernous angiomas is not totally elucidated. The aims of our study were to determine the rate of cerebral hemorrhage, its associated factors, and the clinical outcome in patients with cavernous angiomas in a Hispanic population.

Methods: We studied 133 patients with cavernous angiomas. The patients were classified into two groups depending on whether they presented an ICH. A comparative analysis of demographics and clinical data, neuroimaging characteristics, and prognosis was carried out in patients with and without hemorrhage. The hemorrhage rate (expressed as the percentage per patient per year) was also estimated.

Results: Seventy–eight patients (59%) had hemorrhage. Non–lobar location of angiomas was associated with hemorrhage [OR 4.82 (CI 95% 2.17–10.73; p=<0.001)]. In contrast, factors associated with a decreased risk of hemorrhage were a family history of epilepsy [OR 0.30 (CI 95% 0.10–0.79; p=0.016)] and lobar location of the angiomas [OR 0.21 (CI 95% 0.09–0.46; p=<0.001)]. The hemorrhagic rate of 1.71% per patient per year was influenced by the location. It was only 1.22% per patient per year in lobar angiomas and 2.33, 2.39, and 2.82% per patient per year for brainstem, cerebellum, and deep hemispheric angiomas, respectively.

Conclusions: The non–lobar location of cavernous angiomas gives a higher risk of hemorrhage in our Mexican mestizo population, without the hemorrhage being related to either age or sex.     

伴有周围神经损害的强直性肌营养不良

目的探讨强直性肌营养不良(DM)的临床表现和病理改变。方法通过1例确诊的DM病例的临床表现和神经肌肉的病理改变来系统回顾本病的发病机理、分类、临床表现、病理改变、诊断和治疗。结果该例患者的临床表型和肌肉病理改变符合DM1型,腓肠神经主要病理改变为轴索变性,伴有轻度脱髓鞘。结论DM是一组遗传性疾病,临床表现为骨骼肌萎缩、力弱、强直和多种骨骼肌以外的症状,可伴有以轴索变性为主的周围神经损害。     

Kennedy病的临床、病理及AR基因分析一例

目的探讨Kennedy病(KD)的临床、病理及基因特点。方法对1例KD患者进行临床、电生理和病理检查。抽取该患者及4位家族成员外周静脉血并抽提其基因组DNA,采用PCR-DNA直接测序的方法进行AR基因分析。结果该患者临床表现为缓慢进行性四肢无力,伴有延髓麻痹、肌肉萎缩、肌束跳动、感觉障碍和男性乳房发育;血脂、肌酶升高;肌电图提示前角细胞损害,周围神经感觉及运动传导速度减慢;肌肉病理可见萎缩的肌纤维及肥大固缩的细胞核;AR基因分析发现患者第一外显子CAG重复突变,重复次数为43次,4位家族成员为19～23次。结论该例为散发性KD患者;KD临床表现不典型,肌电图和病理检查提示神经源性损害,确诊需行AR基因分析。     

Spheno-orbital meningioma with unusual radiological features

We report a patient with a spheno-orbital meningioma having unusual clinical and radiological features. Eight days before consultation, this 66-year-old female developed right exophthalmos associated with periorbital pain. Neuroradiological features included, (1) multilocular intratumoral cyst on enhanced CT, (2) preferential extradural growth with prominent bony destruction and intratumoral hemorrhage on MRI, and (3) early venous draining on CAG. Histopathology including immunohistochemistry and electron microscopy revealed typical transitional meningioma. The only uncommon pathological feature was that the attachment consisted of the outer (periosteal) layer of the dura, and the microscopic structure of the inner (meningeal) layer was normal.     

脑出血急性期患者血肿周围细小动脉的病理改变

目的 观察脑出血急性期患者血肿周围脑组织细小动脉的病理改变。方法 43例脑出血患者于急性期行微创血肿清除术,取手术过程中引流出的血肿内脑组织进行HE及甲醇刚果红染色,观察镜下脑组织细小动脉的病理变化。结果 本组有28例（65．12％）脑组织细小动脉的病变基础为细小动脉硬化及玻璃样变,3例（6．98％）可见小动脉粥样硬化,5例（11．63％）可见细动脉内血栓形成;10例（23．26％）脑出血后6h～4d有显著的以中性粒细胞为主的炎细胞黏附及浸润。7例在发病后24h内、1例在24～48h有小灶性出血。结论 脑出血患者最常见的病因为细小动脉硬化;中性粒细胞浸润细小动脉为脑出血急性期最重要的病理改变之一。     

DSA、CT与MRI融合影像对颅内多发假性动脉瘤的诊治价值（附1例报告并文献复习）

目的 报告1例通过DSA、CT与MRI融合影像联合神经导航诊治颅内多发假性动脉瘤患者的手术经验及体会。方法 回顾性分析1例外伤性颅内多发假性动脉瘤患者的临床资料,包括病史、症状、体征、影像学检查、手术过程及病理结果,总结DSA、CT与MRI融合影像对颅内多发假性动脉瘤的诊治价值及临床应用。结果 患者为25岁女性,车祸多发伤入院,经术前DSA检查为左侧大脑前A2段及胼周动脉多发假性动脉瘤,术前经3D-DSA与CT融合影像确定出血责任动脉瘤,经3D-DSA与MRI融合影像解决了精确定位,在神经导航指引下,一期手术完成了两个动脉瘤夹闭并切除。经病理证实为外伤性假性动脉瘤。结论 DSA与CT融合图像可以帮助判定多发动脉瘤中出血的责任动脉瘤,DSA与MRI融合图像输入神经导航为脑深部不能行介入栓塞治疗的颅内动脉瘤开创了新方法。     

Sylvian fissure dermoid cyst with intratumoral hemorrhage: case report

It is rare for a dermoid cyst to develop intratumoral hemorrhage. A 61-year-old woman had a sudden-onset left hemiparesis and slow response to verbal requests for one week when unenhanced computed tomography scanning revealed a mixed iso- and hypo-dense heterogeneous lesion in the right fronto-temporal area. T1-weighted magnetic resonance imaging (MRI) of the brain showed a mixed hyper- and hypo-intense tumor in the right fronto-temporal area. The tumor became hyperintense on T2-weighted MRI and was faintly enhanced at tumor periphery on T1-weighted MRI. The tumor was excised and pathological examination revealed a dermoid cyst with intratumoral hemorrhage. The post-operative course was complicated by hemorrhage in the tumor bed, which was evacuated immediately. The patient improved and could walk without support two weeks after the second operation. After 1 year of follow-up, she was well and without neurological deficits. To the best of our knowledge after a literature review, only two previous cases of dermoid cyst have featured intratumoral hemorrhage.     

Sylvian fissure dermoid cyst with intratumoral hemorrhage: case report

It is rare for a dermoid cyst to develop intratumoral hemorrhage. A 61-year-old woman had a sudden-onset left hemiparesis and slow response to verbal requests for one week when unenhanced computed tomography scanning revealed a mixed iso- and hypo-dense heterogeneous lesion in the right fronto-temporal area. T1-weighted magnetic resonance imaging (MRI) of the brain showed a mixed hyper- and hypo-intense tumor in the right fronto-temporal area. The tumor became hyperintense on T2-weighted MRI and was faintly enhanced at tumor periphery on T1-weighted MRI. The tumor was excised and pathological examination revealed a dermoid cyst with intratumoral hemorrhage. The post-operative course was complicated by hemorrhage in the tumor bed, which was evacuated immediately. The patient improved and could walk without support two weeks after the second operation. After 1 year of follow-up, she was well and without neurological deficits. To the best of our knowledge after a literature review, only two previous cases of dermoid cyst have featured intratumoral hemorrhage.