原发性中枢神经系统血管炎1例报道及文献复习

目的 总结分析原发性中枢神经系统血管炎（PACNS）的临床特点、影像学表现、病理特征、治疗和预后,探讨多学科团队（MDT）模式在中枢神经系统自身免疫性疾病诊疗中的价值。方法 回顾性分析1例PACNS的临床资料,并结合相关文献进行总结。结果 术前初步诊断为右顶叶胶质瘤,在神经导航及荧光引导下完整切除病灶,术后病理检查及MDT讨论明确诊断为PACNS。明确诊断后,给予免疫调节及对症支持治疗,出院后随访6个月,病人症状逐渐恢复正常。结论 PACNS临床诊断困难,易与胶质瘤相混淆,通过MDT讨论,结合颅内血管造影及病理学检查有助于明确诊断。     

原发性中枢神经系统血管炎2例报道并文献复习

目的报道2例原发性中枢神经系统血管炎(PACNS)患者的临床、影像学和病理特点。方法回顾分析2例经病理活检确诊的PACNS患者,总结其临床表现、影像学和病理特点。结果 2例患者均以局灶性神经系统症状起病,以右侧肢体无力伴语言障碍为主要表现,数月内持续进展。2例患者头颅MRI均提示颅内多发水肿病灶伴渗血,增强后有不规则强化,动态MRI随访见病灶均呈持续进展。2例患者血清免疫指标以及血管炎指标均阴性,经脑组织活检病理排除了肿瘤、真菌、结核和病毒感染,证实为PACNS。结论 PACNS以中枢神经系统局限性受损为主要特点,症状常持续性进展,影像学检查有临床诊断意义,但脑组织活检和排除其他疾病是诊断本病的关键。     

表现为复发性视神经炎的肿块样原发性中枢神经系统血管炎一例报道并文献复习

目的加强对肿块样原发性中枢神经系统血管炎(tl-PACNS)的认识。方法回顾性分析1例经病理证实的tl-PACNS患者的临床、影像学、病理表现以及治疗和预后情况。结果患者临床表现为复发性视神经炎,头MRI表现为肿块样长T1、长T2病灶,术后病理表现为软脑膜和小血管壁上淋巴细胞和浆细胞浸润,对糖皮质激素(激素)单用或联合免疫抑制剂治疗有效。结论 tl-PACNS临床表现复杂多样,影像学上极易误诊为肿瘤,脑活检是其确诊的主要依据。tl-PACNS对激素和免疫抑制剂治疗效果好。     

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（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。     

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

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     

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

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。     

Primary central nervous system angiosarcoma: A case report and literature review

Angiosarcoma is a rare vascular malignant neoplasm that mainly occurs in skin and soft tissues. Intracranial localization is very rare and only a few cases have been reported. This report intends to present the clinical, radiological and pathological pictures of a primary central nervous system angiosarcoma along with a review of the literature. A 35‐year‐old woman presented at our institution with weakness and sensory disturbances of her right hand. Neuroimaging revealed a roughly round, hemorrhagic and moderately enhancing lesion in the left frontal posterior region. The tumor was totally removed under awake anesthesia and continuous monitoring of motor and language functions. Histopathology revealed an epithelioid angiosarcoma. Radical removal, followed by adjuvant radiotherapy and chemotherapy, is able to completely control the disease for a relatively long period.     

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS–HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS.In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS–HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy ± chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma.We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS–HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented.     

Primary central nervous system lymphoma presenting with central neurogenic hyperventilation. A case report and review of the literature

INTRODUCTION: Central neurogenic hyperventilation (CNH) in an awake patient is a rare entity. OBSERVATION: We report here a 54-year-old patient who developed central neurogenic hyperventilation as the initial presentation of a primary central nervous system lymphoma located in the brainstem. CONCLUSION: The patient's hyperventilation resolved completely with chemotherapy for primary CNS lymphoma. Most of the cases reported in the literature are related to a diffuse tumor of the brainstem with an intriguing overrepresentation of primary CNS lymphoma. The pathogenesis of CNH is discussed.     

不典型性中枢神经系统Whipple病:一例报告并文献复习

目的结合文献探讨中枢神经系统Whipple病的诊断与治疗特点,以提高对该病的认识。方法回顾分析1例以头痛、左侧肢休无力,伴记忆力减退为首发症状的不典型性中枢神经系统Whipple病的临床诊断与治疗经过,并进行文献复习。结果女性患者,35岁。首发症状表现为头痛、肢体无力及记忆力减退,但不伴发热、癫癎发作。病程进展过程中相继出现阵发性四肢抽动、右侧下肢无力、小便失禁、多食、体质量增加、停经、体温波动,大剂量糖皮质激素及青露素、复方磺胺甲噁唑等抗炎药物治疗无效,随着颅内压逐渐升高,脑疝形成。腰椎穿刺脑脊液检测仅蛋广白定量显著升高。脑电图提示右侧前额颞区慢波。MRI呈以右侧大脑半球、额顶颢叶、半卵圆中心及基底节为主的大片长T₁、长T₂信号,并不均匀疏松团状强化,病灶周围水肿,占位效应明显,并累及左侧大脑半球。病理学检查呈现大片状坏死,脑组织及血管周围大量淋巴细胞和浆细胞浸润,伴大量格子细胞渗出,胞质丰富,内含大量六胺银和PAS染色阳性的细小颗粒状物质。排除中枢神经系统肿瘤、脱髓鞘病变及炎性假瘤等疾病。结论中枢神经系统Whipple病极为罕见,临床及影像学表现复杂多样,病理学检查仅能提示特殊感染,治疗困难,误诊率及病死率高。早期进行组织活检,结合临床表现及病理学特征可以明确诊断,经规范的抗生素治疗,患者可获得良好预后。     

原发性中枢神经系统血管炎1例及文献复习

1 临床资料患者,男,26岁,工程师.因言语不利伴短暂性意识模糊1周于2010年7月10日收治于上海中医药大学附属曙光医院神经内科.患者于1周前剧烈运动后,突感双下肢乏力,并伴有言语不利、短暂性意识模糊及咽痒感,无恶心、呕吐、眩晕等不适症状,即至我院急诊就诊,查血常规示白细胞9.73×109/L,中性粒细胞51.8%,红细胞5.15×1012/L,血红蛋白159.5 g/L,血小板284×109/L.头颅CT平扫未见明显异常.考虑短暂性缺血发作(transient ischemic attack, TIA)合并感染可能,给予抗生素、中药活血化瘀通络等治疗.治疗后,咽痒感症状明显改善,但仍有言语不利及偶发的短暂性意识模糊.入院时,患者诉肢体偶有麻木感,发作性言语不利,舌体僵硬感,无恶心、呕吐、眩晕等不适症状.患者近4月来常有短暂性眩晕感并伴有剧烈头痛发作,可自愈且无后遗症,未进行正规治疗.     

Primary angiitis of the central nervous system: report of five biopsy-confirmed cases from Colombia

INTRODUCTION: Primary (isolated) angiitis of the central nervous system (PACNS) is a rare cause of cerebrovascular disease (CVD), and few leptomeningeal and brain biopsy (LBB)-confirmed cases have been reported from South America. METHODS: We retrospectively reviewed charts of patients with diagnosis of cerebral angiitis admitted between March 1991 and July 2001 to a single university hospital in Medellin, Colombia. Patients with definitive diagnosis of PACNS by Alrawi et al.'s LBB criteria were selected. We excluded other causes of cerebral angiitis as well as cases without LBB confirmation. RESULTS: We report five patients, four men and one woman, with a mean age at onset of 24.4 years, and an average disease progression of 12.4 days. Four presented with headache and motor weakness, three had seizures, and two had alterations of consciousness. Cerebral MRI was abnormal in all five cases; brain CT in four, and cerebral angiography in two. The cerebrospinal fluid (CSF) was abnormal in two patients. Leptomeningeal and brain biopsies revealed mononuclear infiltration in the wall of small blood vessels in all. Three had concurrent meningeal and cerebral involvement, two had necrotizing angiitis, and one had vascular and encephalitic lesions. All received only steroid treatment; the 1-year follow-up revealed good prognosis without relapses. CONCLUSION: We report five biopsy-proven cases of PACNS from Colombia associated with neurological and neuroimaging abnormalities; these patients presented a mild inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid treatment without relapses. Leptomeningeal and brain biopsy is mandatory for a definitive diagnosis.