IgG-index predicts neurological morbidity in patients with infectious central nervous system diseases

Background  

Prognosis assessment of patients with infectious and neoplastic disorders of the central nervous system (CNS) may still pose a challenge. In this retrospective cross-sectional study the prognostic value of basic cerebrospinal fluid (CSF) parameters in patients with bacterial meningitis, viral meningoencephalitis and leptomeningeal metastases were evaluated.     

Primary central nervous system vasculitis in children

OBJECTIVE: Primary angiitis of the central nervous system (PACNS) is a severe and ill-defined neurologic disease. The goal of this study was to characterize the presenting features, treatment, and neurologic outcome of PACNS in children (cPACNS) and to define the predictors of disease progression in order to identify high-risk patients with cPACNS. METHODS: The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical and vascular imaging findings, including identification of arterial stenosis on conventional angiography or magnetic resonance (MR) angiography. Disease progression was defined angiographically at >3 months after initial angiography. Clinical data obtained in prospectively collected standardized assessments and results of laboratory tests, including detection of cerebrospinal fluid abnormalities, were noted, and neuroimaging studies were reanalyzed. Predictors of progression were identified and tested in multivariate regression models. RESULTS: Sixty-two consecutive patients with cPACNS (38 male, 24 female, median age 7.2 years) were included. Two distinct subgroups were identified, those with progressive disease and those with nonprogressive disease. Progressive cPACNS was found in 20 of 62 children and was predicted by a clinical presentation of neurocognitive dysfunction, multifocal parenchymal lesions on MR imaging, and evidence of distal stenoses on angiography. CONCLUSION: The spectrum of PACNS in children includes both progressive and nonprogressive forms. Characteristic features at diagnosis can be used to predict later progression, to identify a distinct high-risk cPACNS cohort, and to help guide selection of patients for immunosuppressive therapy.     

Autoantibodies in inflammatory demyelinating diseases of the central nervous system

The determination of disease-specific autoantibodies (Abs) is a challenge in any autoimmune disease. The significance of Abs detected in inflammatory demyelinating diseases (IDD) of the central nervous system (CNS), such as multiple sclerosis (MS), is still unclear. Histopathological reports have demonstrated that a humoral (Abs)-mediated pattern of demyelination is detected in >50% of MS patients and is consistently associated with active demyelination. The observation that these patients specifically respond to plasmapheresis reinforces the hypothesis of a specific humoral MS subtype. One of the most intensively studied antigen targets in MS is a glycoprotein of the myelin sheath called the Myelin Oligodendrocyte Glycoprotein (MOG). Recent advances have shown that epitope specificity of MOG is crucial in terms of specificity of the Ab response. Several other auto-Abs, including anti-myelin, oligodendrocyte and neuronal Abs have been studied in MS. These auto-Abs may have pathogenic or protective properties, but could also have no functional role. Recently, the demonstration of a highly specific auto-Ab in an IDD of the CNS called neuromyelitis optica (NMO), directed against the aquaporin-4 (AQP-4) located at the blood brain barrier (BBB), has allowed a refinement of the diagnostic criteria of NMO and classification of this disease as an autoimmune channelopathy. These recent advances have reinforced the interest in tracking the role of the humoral response in the different IDD of the CNS.     

Tuberculosis of the central nervous system in preschool children

The clinical and X-ray forms of tuberculosis were studied in 1481 patients aged under 7 years who had been treated from 1970 to 2000. Patients with tuberculosis of the central nervous system accounted for 5.5%. In the past 15 years, there was an increase in the share of generalized tuberculosis, more severity of the clinical course of tuberculous meningitis, and a rise in the incidence of deaths from the disease. Children of the first year of life fell ill more frequently. The main causes should be considered to be exogenous superinfection whose role increases under the conditions of an undetected reservoir of tuberculous infection; no biomedical protection as tuberculosis controlling measures; no protection of children from socially disadapted families. A fatal case was recorded in half the patients. The other half was discharged as having rough residual changes in the central nervous system.     

神经节苷脂GM1与神经系统疾病

神经节苷脂是一类含唾液酸的鞘糖脂,在脑内的含量非常丰富.它不但能够促进神经细胞分化、神经突生长以及突触形成,而且参与了神经可塑性的凋节和脑损伤后的功能恢复.GM1是迄今研究最为深入的神经节苷脂,对细胞内C4~(2+)稳态的调节被认为是GM1神经营养/神经保护作用的基础,而它与神经营养因子的相互作用则是其神经保护作用的关键.此外,它还具有抗兴奋性毒性、抗氧化、扩血管等作用.各种神经变性疾病以及缺血缺氧性脑病均与神经元脱失和凋亡有关,而GM1的神经营养/神经保护作用能在这些疾病的治疗中发挥重要作用.目前,GM1已被广泛用于帕金森病、卒中、新生儿缺血缺氧性脑病、脑外伤、脊髓损伤以及周围神经病的治疗,对其作用机制的进一步研究有望为上述疾病的治疗提供新的思路.     

Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease

Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.     

解耦联蛋白4及其在中枢神经系统疾病中的作用

解耦联蛋白4(uncoupling protein 4,UCP4)是在大脑皮质和海马特异性表达的解耦联蛋白家族成员,可通过解耦联、降低线粒体膜电位、调节Ca2+稳态以及氧化应激等机制在帕金森病、多发性硬化、癫(癎)、脑血管病和脑外伤等中枢神经系统疾病中起着重要作用.文章综述了UCP4及其在中枢神经系统疾病中的作用,以期为开发以UCP4为治疗靶点的药物提供一定的依据.

Abstract：

Uncoupling protein 4 (UCP4) is a member of the multigenic uncoupling proteins (UCPs), specific expressing in the cerebral cortex and hippocampus. UCP4 plays an important role in Parkinson's disease, multiple sclerosis, epilepsy, stroke, brain trauma and other central nervous system diseases by uncoupling, decreasing mitochondrial membrane potential,regulating Ca2+ homeostasis and oxidative stress. This article reviews UCP4 and its roles in the central nervous system diseases in order to provide certain basis for the development of UCP4targeted medication.     

AIDS合并中枢神经系统疾病临床特点分析

目的通过分析AIDS患者合并中枢神经系统疾病的临床特点及诊治情况,以提高AIDS合并中枢神经系统疾病的确诊率和疗效,改善预后。方法回顾性分析北京地坛医院2014年1月—2015年1月住院治疗的62例合并神经系统疾病的AIDS患者临床特点及诊治情况。结果因中枢神经系统疾病人院患者占同期入院患者的9.82%。临床表现主要为发热、头痛、脑膜刺激征阳性、呕吐、肢体运动障碍、意识障碍和头晕等。隐球菌性脑膜炎20例(32.26%);神经梅毒和结核性脑膜炎各8例(12.90%);HIV脑病/进行性多灶性脑白质病5例(8.06%);弓形体脑病4例(6.45%);巨细胞病毒脑炎并神经根炎2例(3.23%);4例经脑活体组织检查,3例分别诊断为化脓性结核性脑膜炎、伯基特淋巴瘤和组织胞浆菌病(各占1.61%),1例病原仍不明;脊髓炎、疱疹病毒脑炎和颅内占位病变各1例(各占1.61%);原因不明中枢神经系统感染9例(14.52%)。经治疗,43例(69.35%)病情好转,10例(16.13%)放弃治疗,5例(8.06%)死亡,无变化4例(6.45%)。结论 AIDS并发中枢神经系统疾病原因复杂,临床确诊困难。发热、头痛、脑膜刺激征阳性、呕吐、肢体障碍和意识障碍为主要临床表现。以隐球菌性脑膜炎发病率最高,其次为结核性脑膜炎和神经梅毒。尽可能查找病原学依据有利于早期明确诊断,改善预后。     

Familial aggregation of multiple myeloma and central nervous system diseases

Degenerative central nervous system diseases such as Alzheimer's disease and lymphoreticular malignancies such as multiple myeloma occur with increased frequency with advancing age. Relatives of early-onset Alzheimer's disease patients may have an increased risk of lymphoreticular malignancies. This led us to evaluate the family history of central nervous system diseases in a case-control study of multiple myeloma. Thirteen of 439 multiple myeloma cases had one or more first-degree relatives with degenerative or demyelinating central nervous system disease. In comparison, there were nine "positive" family histories in 1,317 matched hospital controls (relative risk = 4.4, 95% confidence interval = 1.9-10.3). Relative risks for the component categories of Parkinson's disease, multiple sclerosis, and miscellaneous degenerative central nervous system diseases were 3.0, 4.0 and 11.9, respectively. Our findings suggest that the degenerative and demyelinating central nervous system diseases and the lymphoreticular malignancies may comprise an etiologically related group of "protean diseases." These diseases may have a shared genetic susceptibility, possibly an immunologic abnormality. The varied disease manifestation in family members suggests a second necessary etiologic step of a variable and possibly environmental nature.     

肺炎衣原体与中枢神经系统疾病相关性研究进展

肺炎衣原体(Chlamydia pneumoniae,Cpn)是一类具有独特双相发育周期的专性胞内寄生菌。Cpn感染过程复杂且致病类型广,除引起常见的呼吸道感染外,还可引起多个系统的慢性持续性感染。有文献报道,Cpn与中枢神经系统疾病的发生、发展密切相关。本文对Cpn与中枢神经系统疾病的相关性研究进展进行综述,为今后更合理地指导临床治疗提供重要信息。