原发性中枢神经系统血管炎

原发性中枢神经系统血管炎(pri mary angiitis of the CNS,PACNS)是一种自发、并可复发的局限于中枢神经系统,而没有其它系统性疾病表现的血管炎。它最早由Cravloto和Feign[1]提出,是一种少见的中枢神经系统疾病,临床表现多种多样,实验室检查和影像学表现均无特征性,给诊断带来     

中枢神经系统原发性血管炎的诊断与治疗

目的研究中枢神经系统原发性血管炎(PACNS)的诊断与治疗方法。方法对12例以占位性病变为突出表现的PACNS病人,回顾性分析其临床表现、影像特征及手术治疗结果。结果病变表现为低密度的CT、和长T     

原发性中枢神经系统血管炎的临床、影像及病理学特点

目的探讨原发性中枢神经系统血管炎(PACNS)的临床、影像及病理学特点。方法回顾性分析8例PACNS患者(4例临床拟诊,4例确诊)的临床资料。结果本组男5例,女3例;平均年龄32岁;平均病程11个月。首发症状头痛5例,肢体无力2例,言语不清1例。临床表现头痛8例,肢体无力麻木5例,言语障碍1例,癫癎样发作2例;7例呈缓慢进展病程,1例呈复发、缓解、缓慢进展病程。8例磁共振成像(MRI)均有异常,其中单侧病灶6例,双侧病灶2例;病灶位于脑叶7例,病灶边缘欠清晰、不规则或非典型楔形,同时累及皮质和皮质下,1例病灶位于左额顶部扣带回区,呈大片长条形,病变区外缘呈尖角或指状;病灶均有不同程度的强化,7例沿脑沟脑回强化,1例呈"肿块样"成堆不规则线条状强化;5例弥散加权成像(DWI)呈高信号,表现扩散系数(ADC)为低信号;8例磁共振血管成像(MRA)检查,表现为血管僵硬,血管边缘毛糙不清,管壁欠光滑,节段性狭窄、扩张,末段分支减少;4例全脑数字减影血管造影(DSA)检查,可见脑内动脉中末端呈节段性狭窄、扩张、分支减少。4例脑组织活检,病理结果示以淋巴细胞浸润为主的血管炎。5例行糖皮质激素、3例行环磷酰胺治疗,预后均良...     

原发性干燥综合征中枢神经系统损害八例临床分析

目的探讨原发性干燥综合征的中枢神经系统损害的临床表现、MRI特点、治疗方法。 方法 回顾性分析漳州市福建医科大学附属漳州市医院确诊的8例原发性干燥综合征合并中枢神经系统损害的临床表现、MRI特点。 结果临床可表现为延髓背外侧综合征、肢体无力、智能改变,可类似多发性硬化、视神经脊髓炎,视神经较经常累及;MRI检查示5例出现脊髓损害：病变部位以颈胸髓为主,颈髓2例,胸髓2例,1例同时累及颈髓和胸髓,病灶可大片状、散在斑片状,可轻度、明显强化或不强化,5例出现颅内损害：病变部位可见于延髓背外侧、丘脑、海马、中脑导水管、第四脑室旁、侧脑室旁,可表现为皮质下及脑室旁白质多发小缺血灶,可类似韦尼克脑病,l例出现颈动脉夹层,7例使用了激素后病情有一定改善。 结论原发性干燥综合征中枢神经系统损害的症状、体征及影像学表现多种多样。     

原发性中枢神经系统血管炎研究进展

<正>原发性中枢神经系统血管炎(primary angiitis of the central nervous system, PACNS)是一种罕见重度的累及脑实质、脊髓、软脑膜中小血管的免疫炎性疾病。该病由Harbitz在1922年首次报道,随后20世纪50～70年代陆续有数10篇相关病例的报道,其中绝大多数都是尸检后诊断^[1],在1988年由Calabrese和Mallek正式命名为PACNS。     

原发性中枢神经系统血管炎

原发性中枢神经系统血管炎是中枢神经系统血管的非感染性炎性改变。该病较为少见,病因亦不明确。现对其病因、病理、临床表现、诊断及治疗等方面综述如下。1病因本病病因不明。有作者[1]认为与自身免疫异常有关,可能是T细胞介导的迟发性过敏反应所致。有报道[2]在电镜下可见到患者的单核巨细胞里有类病毒颗粒或类支原体结构,提示可能是感染因素对血管壁造成的直接损伤。也有学者[3]提出抗嗜中性粒细胞胞浆抗体可与嗜中性粒细胞中的颗粒及单核细胞中的溶酶体发生反应,激活体液和细胞的炎性介质导致血管炎。2病理本病组织学表现是多样的,软脑…     

中枢神经系统原发性血管炎的研究进展

中枢神经系统原发性血管炎(PACNS)是一种少见疾病,对该病的诊断存在某些争议,某些研究者提出PACNS诊断需要有尸检或活检的组织学证据,而其他则认可脑血管造影的表现。单纯脑动脉造影、脑脊液(CSF)及MRI检查或联合检查无足够证据证实PACNS诊断的阳性预测值,动脉造影所谓脉管炎的典型表现很可能在活检时不是脉管炎的诊断。临床高度怀疑而活检未证实的病人,还无资料能够证明发生PACNS的证据,也无证明免疫抑制剂治疗的益处,如果病人无PACNS则反而有害。     

原发性中枢神经系统血管炎及诊断商讨

<正>中枢神经系统血管炎可分为:原发性(孤立性):病变原发的仅局限于中枢神经系统的血管炎;继发性:继发于全身感染性或免疫反应性系统性的血管炎。1定义原发性中枢神经系统血管炎(primary angiitis of the central nervous system,PACNS)是一种仅局限于中枢神经系统病因未明,主要侵犯脑实质的中小血管和软脑膜微血管管壁的炎性疾病,也称原发性脑血     

原发性中枢神经系统血管炎的临床特点

目的探讨原发性中枢神经系统血管炎(PACNS)的临床特点。方法对27例PACNS患者的临床资料进行分析。结果PACNS好发于中青年,呈急性或亚急性起病,临床表现以头痛、大脑皮质功能减退、局灶性神经功能缺损及癫疒间样发作为主;头颅MRI示病灶处呈长T1、长T2改变,多有强化;脑组织病理检查示软脑膜血管炎性改变。经糖皮质激素以及抗血小板聚集、清除自由基、降低颅内压、改善微循环等综合治疗,病情明显改善。结论PACNS临床表现多样,综合临床表现、影像学及脑组织活检可作出诊断;应用糖皮质激素治疗可显著提高疗效。     

原发性中枢神经系统血管炎

原发性中枢神经系统血管炎(PACNS)是一种无其他系统性血管炎或不能以其他疾病解释的有中枢神经系统症状的血管炎性疾病。临床表现复杂多样,易与许多疾病相混淆。本文就该疾病的临床特征、病理、诊断、鉴别诊断、治疗及预后进行综述。     

Aseptic meningoencephalitis in primary Sjögren's syndrome

It has been suggested that cerebral vasculitis is the pathogenetic mechanism of the central nervous system manifestations of primary Sjögren's syndrome. We present a fatal case of aseptic meningoencephalitis in an 18-year-old woman with primary Sjögren's syndrome in whom there was no evidence of cerebral vasculitis at autopsy. On two occasions her condition improved markedly following plasmapheresis.     

原发性中枢神经系统血管炎（附8例经病理确诊病例及文献复习）

目的 依据国际通用的诊断标新,分析比较原发性中枢神经系统血管炎（primary c entral n ervous system vasculitis,PCNSV）诊断手段。 方法 收集2012年1月1日-2015年12月31日于首都医科大学附属北京天坛医院神经病学中心神经感染 与免疫科住院治疗的PACNS患者共8例,回顾性分析患者临床资料,总结其临床表现、实验室检查、神 经影像学及神经病理学特征。 结果 临床表现：本组8例患者中,居于前五位的首发症状为慢性头痛、痫性发作、类卒中样局灶 性神经功能缺损、精神行为异常和锥体外系症状,所有患者均未发现特异性的血生化及脑脊液异 常结果,头颅磁共振成像（magnetic resonance imaging,MRI）均可见颅内异常信号,磁共振血管造影 （magnetic resonance angiography,MRA）及数字减影血管造影（digital subtraction angiography,DSA）均未 见明显异常。患者行脑组织活检,病理结果符合PCNSV表现。 结论 PCNSV的诊断要综合考虑患者的临床表现、实验室检查和影像学检查结果,脑组织活检的广 泛开展,有助于提高本病的诊断率。     

中枢神经系统血管炎与头痛

累及中枢神经系统的血管炎被称为中枢神经系统血管炎。头痛是中枢神经系统血管炎 的非特异表现之一,也是其中最常见的神经系统症状。目前按照2012年Chapel Hill会议（Chapel Hill consensus conference,CHCC）血管炎分类标准将血管炎按照受累血管大小不同进行分类。血管炎所致头 痛的发病机制不明,血脑屏障及神经血管单元的破坏在血管炎头痛中可能起到重要作用。不同类型 血管炎累及中枢神经系统的发生率以及头痛的发生时机和特点有差异。识别血管炎相关的头痛,并快 速进行诊断至关重要,以免进行不必要的治疗或导致严重神经系统后遗症。     

9例原发性中枢神经系统血管炎临床、影像及病理特点研究

目的  总结原发性中枢神经系统血管炎临床、磁共振成像（magnetic resonance imaging,MRI）和病理学特点。 方法  收集2012年3月～2014年12月首都医科大学附属北京天坛医院神经病学中心收治的原发性中枢神经系统血管炎患者资料,采用描述性方法对其临床表现、MRI及病理学特点进行分析。 结果  共收集9例患者,其中男性5例（55.56%）,女性4例（44.44%）,年龄范围10～47岁,中位年龄30岁。痫性发作4例（44.44%）,行为认知异常3例（33.33%）,局灶性感觉运动异常5例（55.56%）,头晕2例（22.22%）,钝性头痛2例（22.22%）,面部疼痛1例（11.11%）,视物模糊1例（11.11%）,行走不稳1例（11.11%）。腰穿异常4例（44.44%）。MRI表现为双侧病灶6例（66.67%）,单侧病灶3例（33.33%）,其中受累部位分别为额叶9例（100%）,顶叶5例（55.56%）,颞枕叶4例（44.44%）,合并皮层下白质受累6例（66.67%）,合并脑膜/脊膜受累3例（33.33%）,合并基底节受累1例（11.11%）,合并脊髓受累1例（11.11%）;病变边界不清8例（88.89%）,边界清1例（11.11%）;皮层萎缩伴脑室扩大3例（33.33%）;病灶及脑脊膜强化6例（66.67%）;7例患者行磁共振T2*或磁敏感加权序列（susceptibility weighted  imaging,SWI）,其中病灶表现低信号4例（57.14%）。2例行脑病理学检查,表现为脑实质水肿,血管周围炎性细胞浸润,血管壁坏死和胶质增生。 结论  原发性中枢神经系统血管炎临床表现及影像学多样,病灶合并脑膜和（或）脊膜强化,以及磁共振T2*或SWI序列低信号是其重要影像学特征,脑活检仍是目前重要的确诊手段。     

Nervous system manifestations in Sjögren''s syndrome

Central and peripheral manifestations of the nervous system were evaluated in 48 Sjögren's syndrome patients. Fifty-six percent of the patients had neurological disturbances. The most common manifestations were entrapment neuropathies (19%) and polyneuropathy (15%). Electrophysiological tests gave further evidence of subclinical nervous system involvement in Sjögren's syndrome: electroencephalography (EEG) was abnormal in 48%, and visual evoked potentials (VEP) in 12% of patients tested. To find possible neuropsychiatric abnormalities, the Minnesota Multiphasic Personality Inventory was applied, and 33/43 patients were found to have psychiatric symptoms. The most frequent were depressive symptoms. In 44% of the patients there was additional evidence of extraglandular involvement or autoimmune disorders. No correlation could be found between the groups of patients with or without neurological disturbances in relation to simultaneous occurrence of associated disorders. It is suggested that nervous system involvement in Sjögren's syndrome reflects the pathogenetic consequences of Sjögren's syndrome alone, and not those of associated autoimmune diseases or extraglandular disorders.     

Neurological and neuropsychiatric dysfunction in primary Sjögren's syndrome

In a prospective clinical investigation of 20 patients with primary Sjögren's syndrome (SS), neurological complications, not attributable to other diseases were detected in 14 patients (= 70%). Dysfunction of the peripheral nervous system (PNS) was nearly twice as frequent as central nervous system (CNS) complications. PNS involvement was dominated by symmetric sensory neuropathies, carpal tunnel syndromes, cranial nerve palsies (above all trigeminal sensory neuropathy) and pupillary dysfunction. CNS impairment was represented by cortical atrophy (n = 4), hemiparesis (n = 1) and aseptic meningitis (n = 1). Though CNS complications were rare, psychometric testing revealed diminished cognitive capacity in 14 patients. In addition to the characteristic sicca syndrome patients suffered from musculoskeletal pain and recurring abnormal sensation which frequently lead to the misdiagnosis of functional disorders. Additionally the frequent occurrence of psychiatric symptoms such as nervosity and depression support the impression of a psychosomatic pattern with no organic basis.     

Nervous system involvement in systemic lupus erythematosus, Sjögren syndrome and scleroderma

Introduction. The purpose of this study was to determine, whether there are any differences in the occurrence of nervous system involvement in different systemic rheumatic diseases. The further aim of the present study was to identify and distinguish primary involvement of the nervous system by these diseases and involvement that may be secondary to confounding factors.
Material and methods. The patient population consisted of 122 patients with a connective tissue disease (42 with systemic lupus erythematosus (SLE), 48 with Sjögren's syndrome and 32 with scleroderma). The methods included neurological examination and standard electrophysiological tests.
Results. At least one neurological defect was diagnosed in 69% of SLE patients, in 71% of Sjögren's syndrome patients and in 66% of scleroderma patients. Secondary factors might have contributed to the pathogenesis of neurological symptoms and signs in up to 25–34% of events.
Conclusion. No significant differences were noted in the occurrence of neurological events in patients with SLE, Sjögren's syndrome and scleroderma. The necessity to differentiate between neurological phenomena directly attributed to the systemic rheumatic disease and those which are totally unrelated or secondary events resulting indirectly from involvement of other organ systems is emphasized.     

Vascular Imaging Outcomes of Childhood Primary Angiitis of the Central Nervous System

BackgroundInflammation affecting cerebral blood vessels is a common cause of stroke in children. Arterial abnormalities on vascular imaging are an important risk factor for stroke recurrence. We aimed to describe the vascular imaging outcomes in children with primary angiitis of the central nervous system after 12 months and identify factors associated with vascular progression and stroke recurrence.MethodsWe retrospectively analyzed clinical and neuroimaging data from the BrainWorks Registry of children with large-vessel primary angiitis of the central nervous system. Neuroimaging was collected at baseline and at least 12-month follow-up, and vascular outcome was categorized as improved, stable, or progressed based on comparison of magnetic resonance angiography. Univariate clinical and neuroimaging predictors were associated with outcome by Fisher exact test.ResultsOur study consisted of 27 children; 20 male; median age was 7.92 years (range, two to 15 years). Twelve patients received steroids (44%). Median follow-up time was 16 months (range, 12 to 56 months). Vascular imaging outcome was categorized as improved in 37%, stable in 22%, and progressed in 41% of patients. Discordant progression, defined as progression and improvement occurring simultaneously across multiple vessels, was observed in 26%. Stroke recurred in 15%, occurring exclusively in the group with progression on follow-up imaging (P = 0.02).ConclusionsAfter 12 months, 40% of children with primary angiitis of the central nervous system demonstrated progression on vascular imaging, without apparent clinical or angiographic predictors. Stroke recurrence was associated with vascular progression. Discordant progression is a newly described angiographic finding. Further studies are necessary to determine if this represents a unique characteristic of inflammatory arteriopathies.     

线状硬皮病合并中枢神经系统血管炎

目的 描述线状硬皮病合并神经系统损害病例的临床、影像学和病理学表现,探讨血管病变的病理生理意义。方法 对2例伴有脑损害的线状硬皮病患者进行临床、神经影像学和脑活检病理学评价。结果 1例30岁女性患者出现眼动神经麻痹、性发作和偏瘫,神经影像学发现皮肤病变同侧的颅骨萎缩、脑萎缩、白质病变。1例27岁女性患者出现进行性加重的头痛,影像学发现右侧颞枕部和基底节囊状病变、白质脱髓鞘。二者均存在右额部“类军刀伤”线状硬皮病。脑活检病理学发现：血管壁增厚,纤维素样变性,淋巴细胞浸润,灶状缺血性梗死,白质脱髓鞘。结论 线状硬皮病出现皮肤病变同侧神经系统损害,其机制可能是由于中枢神经系统血管炎导致血液动力学异常所致。