神经精神性狼疮精神症状分析

目的 分析神经精神性狼疮(NPLE)的精神症状特征,以加强精冲科、神经科临床医生对系统性红斑狼疮所致精神症状的理解和认识.方法 对本院2003年4月至2005年11月间收治的104例系统性红斑狼疮(SLE)患者的补体、免疫球蛋白、抗核抗体、抗核糖体P蛋白、抗核糖核酸蛋白抗体、抗脑神经抗体、脑脊液变化及抗双链DNA等实验室指标及出现的精神症状进行回顾性分析.结果 59.62%(62/104)的SLE伴神经精神症状,其中30.77%(32/104)合并有精神症状,表现为类精神分裂症症状者12例;类情感性精神障碍者25例;类神经症症状者17例.精神症状出现时间在发现患SLE后2 d到5年不等,SLE或NPLE中有无精神症状亚组间抗脑神经抗体阳性率差异均有统计学意义(P<0.05),伴有精神症状组较高.结论 在SLE中精神障碍的发生率较高,但症状没有特征性,出现时间变异大,可能与神经免疫损伤机制密切相关,尤其是与抗脑神经抗体相关.     

以延髓梗死为主要症状的狼疮性脑病1例临床分析

<正>系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种临床表现有多系统损害症状的慢性系统性自身免疫病,体内多种免疫调节障碍,产生以抗核抗体为主的大量不同抗体。其中有25%~75%的患者会累及中枢神经系统,以脑组织多见,临床上习惯称之为狼疮性脑病[1],或称神经精神狼疮(neuropsychiatric lupus,NP狼疮)。该病临床表现多样复     

累及中枢神经系统的神经精神狼疮临床分析

研究背景神经精神狼疮临床表现多样,病死率高,诊断与鉴别诊断存在困难。本文探讨累及中枢神经系统的神经精神狼疮患者的临床特点及其与颅内感染性疾病的鉴别诊断。方法回顾分析23例累及中枢神经系统的神经精神狼疮患者的临床表现、多项血清学和脑脊液检查结果、影像学和脑电图等临床资料。结果临床表现为弥漫型神经精神症状者9例、局灶型者14例。血清免疫球蛋白抗核抗体、抗双链DNA抗体、抗Sm抗体、抗核糖体P蛋白抗体、抗SSA抗体和抗SSB抗体阳性检出率分别为21/22、7/22、1/14、2/14、9/14和3/14例,血清补体C3、C4降低者分别为14/20和5/20例;脑脊液白细胞计数和蛋白定量升高者为5/12和7/12例,葡萄糖和氯化物水平降低者为5/12和6/12例。影像学和部分患者(6例)脑电图检查表现异常。结论血清学、脑脊液影像学和脑电图检查均可协助诊断中枢神经系统神经精神狼疮。虽然病程中脑脊液各项指标可或多或少呈现异常,但白细胞计数和蛋白定量升高不明显,葡萄糖和氯化物水平尚在正常值范围。提示中枢神经系统神经精神狼疮患者脑脊液改变不明显,有助于与颅内感染的鉴别诊断。糖皮质激素和免疫抑制剂对治疗中枢神经系统神经精神狼疮有显著疗效。     

狼疮性脑病的脑脊液细胞学表现

狼疮脑(狼疮性脑病)又称作神经精神性狼疮(NPL),是系统性红斑狼疮(SLE)的危重病症,是SLE三大致死原因之一。狼疮脑是由于神经元反应性自身抗体、抗磷酯抗体、免疫复合物等侵犯中枢神经系统所致。临床上主要表现为器质性精神综合征、精神病或精神障碍、癫痫或抽搐、偏瘫等,其中在SLE早期并发狼疮脑的发病率为25%,在治疗过程中可达到60%[1]。有狼疮脑病者均为病情活动者[2]。由于本病临床上表现缺乏特异性,给狼疮脑病的诊断带来一定的困难。一般认为诊断要点为符合SLE诊断标准,且出现神经精神异常。附加以下任何一项即可诊断①脑电图异常,②脑脊液异常,③头颅CT、MRI异常。须排除颅内感染、精神病、高血压病、尿毒症性脑病等影响[3]。在脑脊液的各项检查中,可以表现为:脑脊液压力增高、蛋白增高、细胞数增高(一般不超过500/μl)、糖及氯化物正常或轻度减低,以上各项均缺乏特异性,不能排除其他疾病的影响。随着近年来脑脊液细胞学的发展,显微镜下发现狼疮细胞或狼疮脑病较具特征的吞噬细胞为狼疮脑病的诊断提供了更有力的证据。本实验室观察了自2004年6月至2008年6月共35例临床确诊为狼疮脑病患者的脑脊液,脑脊液细胞学异常者2...     

43例系统性红斑狼疮性脑病病例报告及文献回顾

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种有多系统损害的慢性自身免疫性疾病,患者血清中具有多种自身抗体,其中以抗核抗体为代表。我国患病率为30.13～70.41/10万,其中以20～40岁的育龄女性多见~([1])。若累及中枢神经系统,出现神经精神症状,则称为红斑狼疮性脑病(lupus erythenatosus encephalopathy,SLEE)或神经精神狼疮(neuropsychiatric lupus,NPSLE)。NPSLE具有起病急骤、临床表现复杂多样、诊断困难、治     

以首发神经精神症状的系统性红斑狼疮21例

目的探讨以首发神经精神症状的系统性红斑狼疮(SLE)的临床特点。方法分析21例以首发神经精神症状的SLE的临床表现、辅助检查结果。结果首发精神异常7例(33.3%),偏瘫(脑梗死)6例(28.6%),全面性癫痫发作症状就诊的SLE患者4例(19.0%),急性意识障碍3例(14.3%),四肢弛缓性瘫痪1例(4.8%)。抗双链双螺旋脱氧核糖核酸(ds-DNA)抗体阳性16例(76.2%),抗Smith抗体(抗Sm抗体)阳性7例(33.3%),抗干燥综合征抗原A抗体/抗干燥综合征抗原B抗体(抗SSA/SSB抗体)阳性9例(42.9%),抗核糖体P蛋白抗体(抗r-RNP抗体)阳性10例(47.6%),抗磷脂抗体(APL)阳性7例(33.3%)。首发精神异常的SLE抗r-RNP抗体(85.7%)阳性率高于抗Sm抗体(28.6%)和APL(28.6%),均P0.05。结论 SLE常常以精神症状、神经症状、脑梗死、癫痫、急性意识障碍等发病,误诊为精神类、脑血管疾病等,进行抗核抗体、APL等检查有利于早期诊断。     

鞘内注射联合甲基泼尼松龙冲击治疗神经精神性狼疮72例观察

目的探讨甲氨蝶呤及地塞米松鞘内注射联合甲基泼尼松龙冲击治疗神经精神性狼疮的效果。方法对72例神经精神性狼疮患者应用鞘内注射联合甲基泼尼松龙冲击治疗前后临床表现、头颅CT或MRI及脑脊液分析。结果66例(91.7%)患者神经精神症状得到不同程度缓解。35例(48.6%)患者复查头颅CT或MRI有好转,并且治疗后脑脊液压力下降。结论甲氨蝶呤及地塞米松鞘内注射联合甲基泼尼松龙冲击是针对神经精神性狼疮行之有效的一种治疗方法。     

神经精神性狼疮临床亚型与影像学表现及自身抗体相关性分析

目的神经精神性狼疮(NPSLE)临床表现多样,分为多种亚型。本文总结NPSLE及其亚型的神经影像、血清免疫学表现,探讨临床亚型与影像学表现及自身抗体之间的相关性。方法采集2010年1月1日-2017年12月31日期间首都医科大学宣武医院住院NPSLE患者的临床、神经影像学资料及免疫学指标,总结NPSLE及其各亚型的神经影像、血清免疫学表现,分析NPSLE各临床亚型与特定影像学表现及自身抗体之间的相关性。结果本文34例NPSLE患者临床及影像学表现多样,最常见NPSLE症状为认知障碍(23.5%)、头痛(14.7%);最常见头颅MRI表现为小血管病变(50.0%)及炎性病变(47.1%)。认知障碍及急性精神混乱状态与头颅MRI出现炎性病变显著相关(P0.05);NPSLE急性脑血管病与血清抗磷脂抗体阳性显著相关(P0.05)。结论 NPSLE临床、影像表现多样。认知障碍及急性精神混乱状态与头颅MRI出现炎性病变相关。NPSLE急性脑血管病与血清抗磷脂抗体相关。     

系统性红斑狼疮的神经精神症状

近年来系统性红斑狼疮(S.L.E.)的中枢神经系统(CNS)受累现象引起了人们的注意,而且越来越重视.有人提到S.L.E的CNS受累包括精神病在内者占59%.为了诊断S.L.E.用的一些敏感试验,尤其是去氧核醣核酸(D.N.A)抗体滴定及脑脊液补体水平都有助于更好地认识这种并发病.以精神症状为主的系统性红斑狼疮既难诊断、又不好治疗.作者温习了文献并观察了五例有CNS受累的病例,其结果如后:关于临床表现方面.正如神经梅毒一样,几乎任何神经病性的或精神性的异常都可于S.L.E中见之.神经精神的异常是有联系的,为了清楚起见而分别论述.精神障碍——此或为S.L.E中最普遍的CNS症状.最轻的精神症状即或在已确诊为S.L.E.的患者     

48例临床拟诊为病毒性脑炎的抗NMDAR脑炎10例回顾性诊断分析

目的探究临床拟诊病毒性脑炎患者中N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)抗体的阳性率并总结分析其临床特点。方法荧光免疫法回顾性检测我科过去2 y中48例临床拟诊病毒性脑炎患者脑脊液NMDAR抗体,总结分析NMDAR抗体阳性患者临床资料、影像、电生理及实验室检查等资料。结果 48例临床拟诊病毒性脑炎患者中10例NMDAR抗体阳性(20.8%),修正诊断为抗NMDAR脑炎。男性6例,女性4例,年龄16~70岁。8例有发热、上呼吸道感染或腹泻等前驱感染症状。首发症状中癫痫发作及精神行为异常各有4例,余2例以头痛或意识障碍为首发症状。临床表现与病毒性脑炎类似,主要表现有发热(8例)、精神行为异常(7例)、癫痫发作(6例)、头痛(5例)、意识障碍(4例)及口角及四肢不自主运动(2例)。1例头部磁共振T2及FLAIR像可见边缘叶点片状高信号。9例脑电图不同程度异常。6例脑脊液细胞学示淋巴细胞反应。结论抗NMDAR脑炎临床表现与病毒性脑炎相似,易被误诊。临床上对病因不明的拟诊病毒性脑炎患者应行NMDAR抗体检查,以明确诊断。     

Neuropsychiatric lupus syndromes: relationship with antiphospholipid antibodies

The authors assessed the prevalence of neuropsychiatric manifestations occurring in patients with systemic lupus erythematosus (NPSLE), according to the American College of Rheumatology standardized definitions for NPSLE, and evaluated the relationship between NPSLE and antiphospholipid antibodies. Sixty-one consecutive SLE patients were studied. Neuropsychiatric manifestations consistent with the diagnosis of NPSLE occurred in 44 (72%). Patients with NPSLE showed significantly higher levels of anticardiolipin antibodies.     

神经精神性狼疮的临床表现和MRI特点分析

目的分析神经精神性狼疮(NPSLE)患者常见的临床表现并探讨其头颅MRI的特点及应用价值。方法回顾性分析11例经临床确诊的NPSLE患者的临床资料及头颅MRI检查结果。结果 11例患者中表现为狼疮样头痛8例,癫痫发作4例,言语不利3例,精神症状、视物模糊和偏瘫各2例,意识障碍和共济失调各1例。10例行头颅MRI检查的患者中,有6例出现异常信号影,病灶累及脑叶(额顶枕叶)3例,基底节2例,白质(皮质下白质、脑室旁及半卵圆中心)5例。病灶性质包括腔隙性梗死(4例)、脑血栓形成(3例)和脑萎缩(2例)。结论 NPSLE临床表现复杂多样,以狼疮样头痛及癫痫发作最常见;头颅MRI可发现NPSLE患者脑内病灶的部位及性质,是较为敏感的检查方法,结合临床可以协助诊断。     

Autoantibodies involved in neuropsychiatric manifestations associated with systemic lupus erythematosus: a systematic review

Neuropsychiatric systemic lupus erythematosus (NPSLE) is one of the most important manifestations of SLE, and includes a variety of clinical manifestations, classified by the American College of Rheumatology in 19 different neuropsychiatric syndromes. To date, more than 116 antibodies have been reported in SLE and at least 20 of them, including 11 brain-specific and 9 systemic antibodies, have been controversially associated with NPSLE. To systematically review the available evidence, to define the association between the above antibodies and NPSLE as a whole and with the 19 neuropsychiatric syndromes associated with SLE, by strictly applying the American College Rheumatology case definitions. Medline reports published between 1999 and 2013 investigating the association between antibodies and NPSLE were included. Whenever possible, associations between antibodies and both NPSLE as a whole and with the 19 syndromes were analysed. This systematic review is based on available data from more than 8,000 patients and controls from 42 studies analysing antibodies and NPSLE. Nineteen studies analysed the role of antiphospholipid antibodies (aPL), 11 focused on anti-ribosomal-P protein antibodies and 5 on anti-N-Methyl-d-Aspartate receptor antibodies. Two studies analysed, respectively, antibodies to aquaporin-4 and VH4-34 encoded antibodies. Given the multitude of clinical manifestations related to NPSLE, a single biomarker failed to be reliably associated with all neuropsychiatric events. Our findings provide evidence that aPL, mainly the lupus anticoagulant, and anti-ribosomal P antibodies are significantly associated with specific manifestations of neuropsychiatric disease attributed to SLE, namely, cerebrovascular events and psychosis, respectively.     

New diagnostic criteria for the neuropsychiatric form of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmunologic illness, which apart from changes in the skin, the locomotor system and the internal organs, attacks also the nervous system. The paper presents 19 neuropsychiatric symptomic syndromes which after conduction of multidisciplinary, international research were accepted by the American College of Rheumatology (ACR) as the criteria of systemic lupus erythematosus (SLE). This broadens the criteria applied since 1982, which were only considering acute symptoms and psychoses as characteristic of the neuropsychiatric form of systemic lupus erythematosus (NPSLE). In the new neurologic criteria concerning the CNS are: epileptic attacks and acute attack disorders, headaches, vascular diseases, demyelinating syndrome, aseptical meningitis, chorea, myelopathy. Psychiatric syndromes which make up the new criteria are: acute amentive state, anxiety disorders, cognitive function impairment, affective disorders, psychoses. The criteria connected to the CNS are: cranial nerve damage, mononeuropathy, damage of nerve plexus, polyneuropathy, vegetative neuropathy, myasthenia and acute inflammatory demyelinating polyneuropathy. Clinical symptoms of these syndromes were set and laboratory and visualising tests were developed, which are useful in their diagnosis. The intention of the ACR in setting new, significantly broader criteria of NPSLE, was to stress the diversity of symptoms and for a practical aspect to allow the diagnosis of NPSLE in patients having this disease, in whom the symptoms connected with the nervous system may dominate in the clinical picture, or may be before the dermatological, locomotor or internal organ symptoms.     

The prevalence of neuropsychiatric syndromes in systemic lupus erythematosus

OBJECTIVE: To describe the prevalence of neuropsychiatric (NP) syndromes in a Finnish population of patients with systemic lupus erythematosus (SLE) and to classify them according to the recently developed American College of Rheumatology (ACR) nomenclature and case definitions for NPSLE. METHODS: Cross-sectional, population-based study covering an area with 440,000 people. A total of 58 patients with a definite diagnosis of SLE and aged 16 to 65 years were found in the computerized database of the area hospitals. Of these, 46 (79%) agreed to participate. The diagnosis of various NP syndromes was based on clinical impression (H.A.) following history, examination, review of medical records, and neuropsychologic testing. RESULTS: At least one NP syndrome was identified in 42 patients (91%). The most frequent manifestation was cognitive dysfunction (n = 37; 81%), followed by headache (n = 25; 54%) and mood disorder (n = 20; 43%). When mild NP syndromes (mild cognitive deficit, headache, mild depression, anxiety, electroneuromyography-negative polyneuropathy) were excluded, the prevalence of NPSLE dropped to 46%. CONCLUSIONS: According to the ACR nomenclature, there is a high prevalence of NP manifestations in a population-based sample of patients with SLE. Most NP syndromes were classified as minor; if they were excluded, the 46% prevalence of NPSLE would be slightly less than estimated in previous studies.     

The clinical significance of microembolic signals in patients with systemic lupus erythematosus

Abstract

Background: Microembolic signals (MES) have been found in about 10% of patients with systemic lupus erythematosus (SLE), and have been associated with neuropsychiatric manifestations (NPSLE), including cerebrovascular damage.

Objective: To determine the frequency of MES in patients with SLE, previous and further risk of neuropsychiatric manifestations.

Methods: One hundred and nine consecutive patients with previous diagnosis of SLE and without acute neurological manifestations were evaluated clinically and by transcranial Doppler monitoring (1 hour) from November 2002 to March 2003. The baseline characteristics and outcomes were compared between patients with and without MES. Patients were followed during a mean time of 4·5 years, to detect the appearance of NPSLE, including stroke and transient ischemic attacks.

Results: MES were detected in 16 patients (14·7%; range: 3-64/h); at baseline, previous neuropsychiatric manifestations were more frequent in patients with MES: 12/16 (75%) versus 46/92 (50%) (p = 0·064), especially for cognitive dysfunction (p = 0·036). Antiphospholipid syndrome and cardiovascular risk factors did not differ between groups at baseline and follow-up, neither the proportion of patients on antiplatelet agents. At follow-up, two patients from the MES positive group and six from the MES negative group developed a new neuropsychiatric manifestation (p = not significant).

Conclusion: This study supports an association of MES and a history of NPSLE, especially cognitive dysfunction. MES were not associated with an increased risk of cerebral ischemia and other neurological manifestations at follow-up.     

Different patterns of cerebral perfusion in SLE patients with and without neuropsychiatric manifestations

To investigate brain perfusion patterns in systemic lupus erythematosus (SLE) patients with and without neuropsychiatric systemic lupus erythematosus (NPSLE and non‐NPSLE, respectively) and to identify biomarkers for the diagnosis of NPSLE using noninvasive three‐dimensional (3D) arterial spin labeling (ASL). Thirty‐one NPSLE and 24 non‐NPSLE patients and 32 age‐ and sex‐matched normal controls (NCs) were recruited. Three‐dimensional ASL‐MRI was applied to quantify cerebral perfusion. Whole brain, gray (GM) and white matter (WM), and voxel‐based analysis (VBA) were performed to explore perfusion characteristics. Correlation analysis was performed to find the relationship between the perfusion measures, lesion volumes, and clinical variables. Receiver operating characteristic (ROC) analysis and support vector machine (SVM) classification were applied to differentiate NPSLE patients from non‐NPSLE patients and healthy controls. Compared to NCs, NPSLE patients showed increased cerebral blood flow (CBF) within WM but decreased CBF within GM, while non‐NPSLE patients showed increased CBF within both GM and WM. Compared to non‐NPSLE patients, NPSLE patients showed significantly reduced CBF in the frontal gyrus, cerebellum, and corpus callosum. CBF within several brain regions such as cingulate and corpus callosum showed significant correlations with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and the Systemic Lupus International Collaborating Clinics (SLICC) damage index scores. ROC analysis showed moderate performance in distinguishing NPSLE from non‐NPSLE patients with AUCs > 0.7, while SVM analysis demonstrated that CBF within the corpus callosum achieved an accuracy of 83.6% in distinguishing NPSLE from non‐NPSLE patients. Different brain perfusion patterns were observed between NPSLE and non‐NPSLE patients. CBF measured by noninvasive 3D ASL could be a useful biomarker for the diagnosis and disease monitoring of NPSLE and non‐NPSLE patients.     

Use of aripiprazole in adolescents with a history of lupus-associated psychosis and refractory psychiatric manifestations

Neurologic and psychiatric manifestations are prevalent in children and adults with lupus (labeled by convention neuropsychiatric systemic lupus erythematosus or NPSLE). However, there is a paucity of data on the evaluation and management of NPSLE in youth, with only a few publications describing the use of atypical antipsychotics in children and adolescents with lupus. In children, aripiprazole, a D2/5-HT1A partial agonist, appears to cause less prominent metabolic derangements than other second-generation antipsychotics. This agent may be an important tool in the treatment of pediatric patients with lupus who are at risk for weight gain and dyslipidemia due to disease and corticosteroid effects. The authors present two cases in which psychiatric symptoms associated with treatment-refractory lupus responded to aripiprazole pharmacotherapy.     

Prevalence and pattern of cognitive impairment in systemic lupus erythematosus patients with and without overt neuropsychiatric manifestations

The prevalence and pattern of cognitive impairment in systemic lupus erythematosus (SLE) patients with (NPSLE) and without (nSLE) overt neuropsychiatric manifestations were investigated. Fifty-two nSLE patients, 23 NPSLE patients and 27 healthy controls were evaluated with a battery of standardized neuropsychological and psychological tests. Disease duration, disease activity index, and current corticosteroid therapy were collected. Cognitive impairment was identified in 14 (26.9%) and in 12 (52.2%) of subjects with nSLE and NPSLE, respectively. Both SLE groups showed a significant impairment compared with controls on tasks assessing verbal and non-verbal long-term memory, and visuoconstructional abilities. In addition, NPSLE patients reported worse performances than both nSLE patients and controls on task evaluating short-term visuospatial memory. NPSLE subjects were significantly more anxious and depressed compared to both nSLE subjects and controls. By multivariate analysis, only depression levels, among clinical variables, significantly predicted cognitive performance. This study shows that cognitive impairment occurs frequently in both nSLE and NPSLE subjects. The higher frequency in NPSLE may be related to coexisting depressive disturbances.