原发性中枢神经系统血管炎1例临床报告

<正>原发性中枢神经系统血管炎(primary angiitis of the central nervous system,PACNS)是发病原因不清、临床表现复杂的非特异性疾病,曾被称为孤立性中枢神经系统血管炎、肉芽肿性血管炎等。平均发病年龄在40多岁(3～74岁),男     

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。     

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…     

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。     

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的临床及病理学特点(附1例报告)

目的 探讨原发性中枢神经系统血管炎(PACNS)的临床及病理学特点.方法 回顾性分析1例PACNS患者的临床资料.结果 该患者临床表现为癫(癎)发作及左侧肢体无力;头颅MRI显示右额顶叶长T1、长T2异常信号;脑活检可见小动脉炎性细胞浸润,管腔狭窄.糖皮质激素及环磷酰胺治疗6个月,临床表现及MRI未见明显改变.结论 PACNS的临床表现无特异性,脑组织活检对诊断具有重要意义.     

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与影像学特征

目的：探讨原发性中枢神经系统血管炎的临床和影像学特征。方法：报道1例23岁男性原发性中枢神经系统血管炎患者的临床、实验室检查和影像学资料。结果：本例患者有反复发作的头痛、多灶性神经功能缺损、认知功能下降等表现以及复发和渐进病程，临床和辅助检查排除了系统性血管病和其他中枢神经系统疾病，头颅MRI显示颅内多灶性和弥漫性病变，MRA和DSA显示右侧大脑中动脉狭窄。结论：原发性中枢神经系统血管炎需通过临床表现、实验室检查和影像学检查排除相关疾病后诊断。     

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。     

A case of childhood unilateral relapsing primary angiitis of the central nervous system

The patient was a 17-year-old girl with transient right-sided weakness and dysesthesia associated with headache and nausea. Head magnetic resonance imaging (MRI) revealed white matter lesions confined to the left hemisphere. Initially, multiple sclerosis was suspected, and methylprednisolone (mPSL) pulse therapy was administered, followed by fingolimod hydrochloride. However, on day 267, the patient again experienced transient hypesthesia. Cranial MRI showed expansion of the highly infiltrated areas of the left hemisphere on fluid-attenuated inversion recovery (FLAIR) and T2 weighted image, accompanied by edema. Multiple contrasting areas were also observed. Susceptibility-weighted imaging demonstrated several streaks and some corkscrew-like appearances with low signals from the white matter to the cortex, suggestive of occluded or dilated collateral vessels. Multiple dotted spots indicating cerebral microbleeds (MBs) were also observed. A brain biopsy revealed lymphocytic, non-granulomatous inflammation in and around the vessels. Vascular occlusion and perivascular MBs were prevalent. The patient was diagnosed with relapsing primary angiitis of the central nervous system (PACNS), and immunosuppressive treatment was initiated, mPSL 1000 mg/day pulse therapy. The patient's clinical symptoms and neuroradiological abnormalities gradually improved. She is now receiving oral prednisolone (6 mg/day) and mycophenolate mofetil (1750 mg/day). This case corresponds to unilateral relapsing, which has recently been reported as a specific clinicopathological subtype of PACNS.     

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...     

Primary angiitis of the central nervous system: report of eight cases from Southern China

Background and purpose:  This study is to report the clinical, neuroimaging and pathological characteristics of patients with primary angiitis of the central nervous system (PACNS) from Southern China.
Methods:  Eight patients with PACNS admitted between August 1995 and April 2006 were retrospectively studied. Records of clinical features, neuroimaging, brain biopsy and therapy were analyzed.
Results:  Primary angiitis of the central nervous system occurred predominantly in youth and middle-aged adults. Headache, hemiplegia and speech disturbance were the most predominant manifestations. The cerebrospinal fluid (CSF) was slightly abnormal in only one case, MRI was abnormal in seven, magnetic resonance angiography in seven, diffusion-weighted imaging in five and digital subtraction angiography in four. Brain biopsy in four cases revealed lymphocytic angiitis. All cases had good outcome with the treatment of single corticoid or cyclophosphamide.
Conclusion:  We report eight cases of PACNS from Southern China associated with neurological and neuroimaging abnormalities; these patients presented a mild to moderate inflammatory disease that was correlated with few CSF abnormalities and good response to single steroid or cyclophosphamide treatment without relapses. Although brain biopsy represents the gold standard for diagnosis of PACNS, considering the difficulty and challenge of identification of this disease, combination with other examinations might be necessary to arrive at an early and definitive diagnosis.     

B‐cell dominant lymphocytic primary angiitis of the central nervous system: Four biopsy‐proven cases

We report four cases of biopsy‐proven B‐cell‐rich primary angiitis of the central nervous system (PACNS). The mean age of the patients was 29 years (range, 23–37 years). The patients suffered from unilateral weakness (n = 2), seizure (n = 1), and hypersomnia, anorexia and confusion (n = 1). The vital signs and the results of laboratory tests were within normal limits in all the four cases except erythrocyte sedimentation rate (ESR) and C‐reactive protein (CRP). ESR was elevated in one patient and CRP was elevated in two patients. The magnetic resonance imaging (MRI) scans revealed single (n = 2) or multiple (n = 2) irregularly enhancing lesions. Radiological studies initially indicated tumors such as glioma (n = 2) or lymphoma (n = 1), except in one case, in which the radiological analysis indicated vasculitis or demyelinating disease. All the cases involved both medium‐sized (50–250 µm in diameter) and small‐sized vessels (20–49 µm in diameter). The vascular, perivascular and parenchymal lymphocytes were polymorphous; however, CD20‐positive B‐cells were predominated in blood vessels while the CD8‐positive T‐cells infiltrated predominantly in brain parenchyma. Therefore, our patients revealed B‐cell dominant lymphocytic vasculitis. Two patients who underwent active treatment (corticosteroid alone or with cyclophosphamide) showed remarkable clinical and radiological improvement but two patients still have initial neurological symptoms, namely, confusion and newly developed seizures, respectively, during the 19–101‐month follow‐up periods; this effect can be attributed to irreversible brain damage. Therefore, although early brain biopsy may be associated with histopathologic diagnostic pitfalls, it is a mandatory procedure for obtaining a confirmative diagnosis as well initiating early therapy, thereby reducing brain damage.     

误诊为脑内多发转移癌的中枢神经系统原发性血管炎的临床分析-附2例报道

目的:探讨误诊为颅内多发转移癌的中枢神经系统原发性血管炎Cpri mary angiitis of central nervous sys-tem,PACNS)的临床特点,提高临床医生对该病的认识水平。方法:报告了2例初步诊断为"脑内多发转移癌"的病人,进行临床特征、影像学资料分析及活组织检查,病理证实为PACNS。并对PACNS的相关文献进行复习。结果:报道的2个病例符合PACNS的特点。PACNS是一种少见的中枢神经系统疾病,其发病机制目前尚不完全清楚。大部分PAC-NS病人的病情都很严重,患者颅内病变累及整个皮层和白质,很快出现大脑皮层高级功能减退等症状,病灶的不规则环形强化,环形强化表面可辨皮层的沟回影像是一些PACNS患者的特征性表现。脑及脑膜的活检仍是诊断PACNS的金指标。结论:当脑内多个病灶,出现大脑皮层高级神经活动症状,未检测到原发病灶,不要轻易诊断为"脑内多发转移癌",要进行脑组织的活检,以除外PACNS这种罕见疾病的诊断,否则就会误诊和漏诊     

IVIG in childhood primary angiitis of the central nervous system: A case report

Aggressive immunosuppressive therapies have been proposed to treat primary angiitis of the central nervous system (PACNS). Here, we report the first successfully stabilized case of childhood, small-vessel PACNS with intravenous immunoglobulin (IVIG) therapy.A 12-year-old boy was admitted to our hospital complaining of recurrent headaches and upper-left homonymous quadrantanopia, since the age of 11 years. Brain computed tomography scans revealed fine calcification in the right temporal and occipital lobes. Brain magnetic resonance imaging scans revealed white matter lesions, with gadolinium enhancement, which waxed, waned, and migrated for 1 year, without immunomodulatory therapies. A cerebrospinal fluid study showed pleocytosis (12 cells per µl). No clinical or serological findings suggested systemic inflammation or vasculitis. Brain angiography was unremarkable. Brain biopsy revealed thickened and hyalinized small vessels, with intramural infiltration of inflammatory cells, which confirmed the diagnosis of small-vessel PACNS. Because the patient developed surgical site infection following biopsy, the administration of monthly IVIG (2 g/kg) was prescribed, instead of immunosuppressive agents. After IVIG therapy, the patient remained stable, except for a single episode of mild radiological exacerbation at 16 months, which occurred when the IVIG interval was expanded. Oral prednisone was added and gradually tapered. At 50 months, his intellectual abilities and motor functions were normal, although he showed residual upper-left homonymous quadrantanopia and post-exercise headache. A temporary headache, associated with the immunoglobulin infusion, was resolved by slowing the infusion rate. PACNS should be treated aggressively to improve prognosis. However, when immunosuppressants are contraindicated, IVIG may be an alternative therapeutic option.     

The activation pattern of macrophages in giant cell (temporal) arteritis and primary angiitis of the central nervous system

To determine if the pattern of macrophage activation reflects differences in the pathogenesis and clinical presentation of giant cell arteritis and primary angiitis of the central nervous system, specimens of 10 patients with giant cell arteritis and five with primary angiitis of the central nervous system were immunohistochemically studied and the expression of the macrophage activation markers 27E10, MRP14, MRP8 and 25F9 was determined in the vasculitic infiltrates. Thus, a partly different expression pattern of macrophage activation markers in giant cell arteritis and primary angiitis of the central nervous system was observed. The group comparison revealed that giant cell arteritis cases had significantly higher numbers of acute activated MRP14‐positive macrophages, whereas primary angiitis of the central nervous system is characterized by a tendency toward more MRP8‐positive intermediate/late activated macrophages. Furthermore, in giant cell arteritis comparably fewer CD8‐positive lymphocytes were observed. These observations suggest, that despite their histopathological similarities, giant cell arteritis and primary angiitis of the central nervous system appear to represent either distinct entities within the spectrum of granulomatous vasculitides or different stages of similar disease processes. Their discrete clinical presentation is reflected by different activation patterns of macrophages, which may characterize giant cell arteritis as a more acute process and primary angiitis of the central nervous system as a more advanced inflammatory process.     

瘤样原发性中枢神经系统血管炎的诊治分析（附6例报道并文献复习）

目的 探讨瘤样原发性中枢神经系统血管炎（TLP-PACNS）的诊断、治疗及预后,以提高对该病的认识。方法 回顾性分析2011年3月～2021年2月手术治疗的6例TLP-PACNS的临床资料,并对相关文献进行复习。结果 术前头颅MRI显示单发局限性病灶3例,考虑胶质瘤;多发病灶3例,考虑转移瘤。6例均行开颅病灶切除术,术后病理证实为PACNS。2例术后给予激素治疗后明显好转;1例多发病灶术后激素治疗后无变化,加用环磷酰胺后好转;3例单发病灶术后自然缓解;随访3个月～2年无复发。结论 TLP-PACNS是一种非常少见的疾病,临床表现和影像学表现缺乏特异性,术前易误诊,病理检查是诊断金标准,激素治疗是一种有效的方法。     

24例原发性中枢神经系统血管炎患者的MRI表现

目的观察24例原发性中枢神经系统血管炎(PACNS)的磁共振成像(MRI)表现,探讨PACNS的MRI诊断价值。方法对急性期进行了MRI检查的24例经病理证实的PACNS的MRI特点进行了回顾性分析。结果 24例患者中,病灶以幕上多见[19例(79.2%)],最常累及颞叶、额叶、顶叶及基底节区(包括丘脑),分别为13例(54.2%)、10例(41.7%)、8例(33.3%)、8例(33.3%);大部分病灶累及双侧大脑半球[15例(72.5%)],灰质和白质均受累[21例(87.5%)]。MRI上病灶可多发或单发,均为12例(50%)。病灶形态大致可分为四种类型:斑片状、肿块样、脑回状和混合性,分别为12例(50%)、8例(33.3%)、2例(8.3%)和2例(8.3%)。MRI上所有病灶均呈长(稍长)T1WI、长(稍长)T2WI异常信号,增强扫描可见病灶均有强化,FLAIR上均呈高或稍高信号,9例(37.5%)病灶中心呈散点状短T1WI、短T2WI混杂信号。17例行DWI和ADC序列检查者中,9例(52.9%)在DWI上呈高(稍高)信号,ADC上呈等信号或混杂信号影,8例(47.1%)在DWI及ADC上呈等信号。11例行GRE序列检查者,8例(72.7%)可见病灶局部有点状低信号影或病灶周围血管影增粗,余3例未见异常。8例行MRA序列检查者,仅1例发现异常,可见病灶供血区相应血管局部有狭窄。结论本组24例PACNS患者急性期头MRI均有异常,表现多样,病灶以幕上多见,可累及各个脑区,病灶可多发或单发,多数灰白质均受累。其形态可表现为斑片状、肿块样、脑回状或混合性,增强扫描均可见强化,以斑片状或脑回样强化多见。头MRI上病灶多变、灰白质受累、斑片状或脑回样强化等表现对PACNS诊断有一定提示性。