系统性红斑狼疮脑病的临床和MRI表现

系统性红斑狼疮 (SLE)病程中常出现神经系统损害 ,其中以脑实质损害为主 ,临床称之为系统性红斑狼疮脑病。本文报道临床确诊系统性红斑狼疮脑病同时行脑 MRI检查患者 2 0例临床和 MRI表现回顾性分析结果。1　临床资料1 .1　一般资料 :2 0例均为女性 ,年龄 1 5～ 4 0岁 ,平均 2 6.4岁 ,病程 1月～ 8年不等。全部病例符合 1 987年中华医学会风湿病学会关于 SLE的诊断标准。1 .2　临床表现 :兴奋、多语 1 1例 ,抑郁 4例 ,偏瘫 7例 ,癫痫发作 9例 ,血管性头痛 3例 ,意识障碍 2例 ,共济失调 1例 ,失语 1例 ,颅神经麻痹 1例。1 .3　脑 MRI表…     

系统性红斑狼疮合并中枢神经系统感染34例临床分析

目的研究分析系统性红斑狼疮(SLE)合并中枢神经系统感染的临床表现。方法回顾性分析2001年1月至2011年12月石家庄平安医院34例SLE合并中枢神经系统感染患者临床资料,提出临床特征及其相关危险因素。结果 34例SLE合并中枢神经系统感染,发生率为1.44%;其中结核性脑膜炎25例(73.53%),新型隐球菌脑膜炎7例(20.59%),病毒性脑膜炎2例(5.88%)。给予内科常规治疗,25例结核性脑膜炎痊愈21例,死亡4例;7例隐球菌脑膜炎痊愈3例,死亡4例。病毒性脑膜炎痊愈1例,死亡1例。本文SLE合并中枢神经系统感染34例中总共死亡9例(26.47%)。全部患者在诊断前均经过糖皮质激素治疗,28例患者应用较大剂量激素治疗,18例患者联合免疫抑制剂治疗。SLE合并中枢神经系统感染的临床特点主要为高热、头痛及意识障碍;狼疮活动度评分(SLEDAI)平均9±3分。脑脊液检查发现糖和氯化物降低明显。结论①系统性红斑狼疮合并中枢神经系统感染以结核性脑膜炎最常见;②肾上腺皮质激素、免疫抑制剂的应用是SLE合并感染的危险因素;③SLE合并中枢神经系统感染与狼疮活动度无关,而神经精神狼疮多发生于狼疮高度活动期,SLEDAI评分对二者鉴别诊断有意义;④狼疮合并中枢神经系统感染以高热、头痛及颅内压增高为主要表现,反复腰穿脑脊液检查对SLE合并中枢神经系统感染的诊断和判断预后有重要价值。     

系统性红斑狼疮脑病脑部病变影像学表现

目的 总结系统性红斑狼疮脑病(systemic lupus erythematosus encephalopathy,SLEE)影像学表现,为系统性红斑狼疮脑部病变的影像诊断提供依据.方法 回顾性分析16例神经系统性红斑狼疮患者脑部CT和MRI表现.结果 6例系统性红斑狼疮脑炎表现为双侧大脑半球白质区较对称性异常信号,1例表现为脑萎缩.4例脑梗死表现为基底节区和额顶叶多发密度或(和)信号异常,增强T1WI无明显强化或环状高信号.1例表现为基底节区较对称钙化影.2例表现为多灶性白质脱髓鞘样改变.1例合并霉菌感染表现为脑干信号异常,增强T1WI呈环状强化.1例霉菌性脑膜炎和1例单纯头痛患者,影像学无异常表现.结论 影像学检查可以发现大部分SLEE脑部病变,并可根据影像学表现和临床症状及相关实验室检查作出定性诊断.     

系统性红斑狼疮累及中枢神经系统的临床分析(附93例报告)

目的对系统性红斑狼疮(SLE)的中枢神经系统(CNS)病变特点及临床预后进行总结.方法对93例伴有中枢神经系统损害的SLE患者进行回顾性分析.结果①SLE的CNS损害症状可分为以下几类弥漫性症状(40.9%)、局灶性症状(23.7%)、抽搐(24.7%)、头痛(10.8%).②局灶性症状的患者预后明显差于弥漫性症状者,抽搐和头痛患者的预后与弥漫性症状患者类似.③脑脊液蛋白增高及CNS损害发生早的患者预后差.结论本组SLE患者CNS受累的症状分布与国外报道类似,不同症状患者的预后情况有明显差异.     

系统性红斑狼疮合并脊髓病变6例临床分析

目的初步探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)合并脊髓病变的临床特征和诊治方法。方法收集SLE合并脊髓病变患者6例,分析其发病时间、临床表现、影像学和脑脊液(CSF)特征以及治疗和转归。结果临床均表现为受损平面以下截瘫,感觉障碍和括约肌功能异常。其中1例以脊髓损害(合并脑膜炎)为首发症状,余5例于狼疮活动期出现脊髓症状。脊髓MRI检查显示不同节段脊髓长T1长T2信号,病变主要位于胸段脊髓,累及节段较长。行CSF检查显示,CSF蛋白升高5例,寡克隆区带(oligoclonal band,OB)阳性3例,白细胞不同程度增加2例。5例给予甲基泼尼松龙冲击 环磷酰胺治疗,另1例仅予激素(地塞米松)治疗,结果显示,2例CSF恢复正常且临床症状好转,1例CSF恢复正常但症状无好转,1例临床症状好转但CSF蛋白进行性增高且有梗阻现象,2例死亡。结论SLE合并脊髓病变常出现在狼疮活动和/或减药过程中,也可作为SLE的首发症状;通过临床表现难以与其他原因引起的脊髓损害鉴别;预后不佳,系统而规范地治疗SLE值得重视。     

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。     

以帕金森综合征为主要表现的系统性红斑狼疮性脑病(附2例报告)

系统性红斑狼疮(SLE)是一种可累及全身各种组织及脏器的自身免疫性疾病,出现中枢神经系统病变称为狼疮性脑病(NPLE)。以帕金森综合征为主要临床表现的NPLE罕见, 现报告2例如下。 1 病例 1．1 例1,男,18岁。因反复发热2个月,四肢无力、张口吞咽困难9天,于2005年3月20日入院。入院前2个月反复发热,体温39 C-39．5 C,伴有脱发、口腔溃疡、关节疼痛,9 天前出现四肢无力,不能行走,张口吞咽困难。既往健康。查体:神志清楚,情感淡漠,沉默少语．语速缓慢,语音低微且含     

系统性红斑狼疮并发中枢神经系统病变患者的血小板活化分析

目的 对系统性红斑狼疮(SLE)并发中枢神经系统病变患者进行血小板活化检测,探讨其可能发病机制. 方法 回顾性分析45例系统性红斑狼疮并发中枢神经系统病变患者的CD62P (P选择素)表达. 结果 弥漫性病变及局灶性病变组的CD62P阳性率均较对照组显著增高,差异具有统计学意义(P＜0.01),而局灶性病变组的CD62P阳性率明显高于弥漫性病变组(P＜0.05).结论 血小板大量活化引起血流动力学异常及脑血管病变可能是系统性红斑狼疮并发中枢神经系统病变的发病机制之一.     

系统性红斑狼疮脑病

系统性红斑狼疮脑病孙淑清林仲玉黄丽红系统性红斑狼疮（ＳＬＥ）神经系统损害侵及脑者最为多见，临床上通称为ＳＬＥ脑病。现将我院自１９７６～１９９５年收治的３０例患者报道如下。资料：本组男１例，女２９例。年龄９～５２岁，平均２７．５岁。４０岁以下２６例，４...     

老年人脑白质病变的临床MRI研究

目的 :探讨老年人脑白质病变 (White Matter L esion,简称 WML )的 MRI表现及其临床意义。方法 :采用 MRI SE序列对 82例老年人 WML进行颅脑 MRI检查 ,评估其 MRI表现及其临床意义。结果 :老年人 WML的MRI主要表现是脑白质内点状及斑片状信号异常 ,以 T2加权像改变为明显。病变分为腔隙性脑梗塞或 /和腔隙病变、脑白质稀疏症、皮层下动脉硬化性脑病 (subcortical arterosclerotic encephalopathy,简称 SAE)等几种类型。 WML病变常见于患有高血压和高脂血症的患者 ,临床上可无症状 ,也可表现为神经、精神的异常。结论 :老年人 WML以腔隙性脑梗塞和腔隙病变最多见 ,SAE少见 ,而脑白质稀疏症多见于无症状的老年人     

Possible Induction of Systemic Lupus Erythematosus by Valproate

Two patients developed clinical and laboratory evidence of systemic lupus erythematosus (SLE) during treatment with valproate (VPA) preparations. The first patient, a 47-year-old man, had fever, malaise, and thrombocytopenia 1 month after VPA was added to phenytoin (PHT) and primidone (PRM). He developed high titers of antinuclear antibodies (ANA) and anti-DNA antibodies, and hypocomplementemia. After discontinuation of PHT and VPA, steroid and immunoglobulin treatment was required for 4 weeks before his condition improved. The second patient, a 28-year-old woman, had been followed for idiopathic leukopenia for 3 years and had previously experienced fever and lymphadenopathy from PHT. After 4 months of divalproex therapy, she developed confusion, joint pain, and a dramatic increase in seizure frequency. She also developed high titers of ANA and anti-DNA antibodies and hypocomplementemia, along with a further decrease in white blood cell (WBC) count. These responded to steroid therapy and withdrawal of divalproex. Three months later, reintroduction of divalproex was followed by a return of ANA in low titer, which resolved after discontinuation. We believe that VPA may have caused true SLE in these patients, one of whom was probably predisposed.     

Systemic Lupus Erythematosus Associated with Use of Valproate

Summary: A 30-year-old man with long-standing localization-related epilepsy and mental retardation had seizures that were partially controlled with valproate (VPA) 500 mg four times daily. Routine examination showed severe thrombocytopenia with mild leukopenia and chronic lowgrade hemolytic anemia. Pertinent laboratory results included positive ANA, rheumatoid factor, anti-NIA, circulating immune complexes, and antihistone antibody. The patient was treated with high dosage prednisone with partial improvement, but continued to have exacerbations at lower dosages. Fourteen months later, VPA was discontinued, and rapid improvement ensued. Prednisone was subsequently discontinued, and the patient has now maintained normal platelet counts for 18 months.     

Valproate-Induced Systemic Lupus Erythematosus in a Patient with Partial Trisomy of Chromosome 9 and Epilepsy

Summary: We report a mentally retarded 30–year-old woman with partial trisomy of chromosome 9 (46,XX6,+der(6)t(6,9)pat) who has had epilepsy since age 11 months. She had been treated with various combinations of drugs. After 1 year of treatment with valproate (VPA) and ethosuximide (ESM), the patient developed arthral-gias, muscle weakness, fatigue, and fever. Laboratory examination showed increased sedimentation rate, hyper-gammaglobulinemia, and high titers of antinuclear antibodies (ANA). The possibility of VPA-induced systemic lupus erythematosus (SLE) was considered. This diagnosis was supported by detection of antihistone antibodies and the HLA-DR4 antigen. VPA dosage was tapered and discontinued, with accompanying resolution of clinical, immunological and hematological signs of SLE 6 weeks after VPA discontinuation. This is the fourth reported case of VPA-induced SLE.     

系统性红斑狼疮合并周围神经病的临床特点和肌电图改变

分析２２例临床有或疑有神经系统损害的系统性红斑狼疮（ＳＬＥ）患者，其中仅３例被临床诊断为ＳＬＥ合并周围神经病，然神经电生理检查证实１３例为周围神经损害。主要临床特点为对称性和非对称性四肢远端麻木、疼痛，深浅感觉障碍，肌力减退，肌萎缩等。神经电生理检查显示神经传导速度减慢，波幅降低，异常自发电位（纤颤电位和正锐波），运动单元多相电位增加。电生理检查与病理改变相符：ＳＬＥ合并周围神经病既有轴索损害，又有脱髓鞘改变。提示神经电生理检查可以为ＳＬＥ患者提供早期或亚临床周围神经损害的依据。     

Suppression of water intake by immune complex formation in the hypothalamus : Implications for Systemic Lupus Erythematosus

Immune complex formation in the perifornical region of the hypothalamus resulted in depressed water consumption in rats, but did not consistently alter body temperature. The antibody with an unrelated antigen did not affect water consumption or body temperature. These results support the notion that immune complex reactions with the central nervous system can alter behavior.     

Cerebral Venous Sinus Thrombosis as a Rare Complication of Systemic Lupus Erythematosus: Subgroup Analysis of the VENOST Study

AimSystemic lupus erythematosus (SLE) is an unusual risk factor for cerebral venous sinus thrombosis (CVST). As few CVST patients with SLE have been reported, little is known regarding its frequency as an underlying etiology, clinical characteristics, or long-term outcome. We evaluated a large cohort of CVST patients with SLE in a multicenter study of cerebral venous thrombosis, the VENOST study, and their clinical characteristics.Material and MethodAmong the 1144 CVST patients in the VENOST cohort, patients diagnosed with SLE were studied. Their demographic and clinical characteristics, etiological risk factors, venous involvement status, and outcomes were recorded.ResultsIn total, 15 (1.31%) of 1144 CVST patients had SLE. The mean age of these patients was 39.9 ± 12.1 years and 13 (86.7%) were female. Presenting symptoms included headache (73.3%), visual field defects (40.0%), and altered consciousness (26.7%). The main sinuses involved were the transverse (60.0%), sagittal (40.0%), and sigmoid (20.0%) sinuses. Parenchymal involvement was not seen in 73.3% of the patients. On the modified Rankin scale, 92.9% of the patients scored 0-1 at the 1-month follow-up and 90.9% scored 0-1 at the 1-year follow-up.ConclusionsSLE was found in 1.31% of the CVST patients, most frequently in young women. Headache was the most common symptom and the CVST onset was chronic in the majority of cases. The patient outcomes were favorable. CVST should be suspected in SLE patients, even in those with isolated chronic headache symptoms with or without other neurological findings.