特异性神经元抗体对神经系统副肿瘤综合征筛检价值的研究

目的　探讨特异性神经元抗体 (尤其是抗 Hu抗体 )对国内人群神经系统副肿瘤综合征 (paraneoplasticsyndromesofnervoussystem ,PNSNS)筛检诊断的特异性和灵敏性。方法　采用免疫组织化学ABC法和Westernblot对 2 0例PNSNS和 12 0例非PNSNS患者的血清进行检测 ,并采用流行病学筛检公式计算出特异度和灵敏度。结果　PNSNS组和非PNSNS组ABC法阳性结果差异有统计学意义 (χ2 =33 4 5 ,P <0 0 1) ,特异性神经元抗体筛检诊断PNSNS的灵敏度为 95 % ,特异度为 72 5 % ;PNSNS组和非PNSNS组Westernblot法阳性率差异有统计学意义 (χ2 =117 12 ,P <0 0 1) ,抗 Hu抗体筛检诊断的PNSNS的灵敏度为 95 % ,特异度为 98 3%。结论　血清特异性神经元抗体 (尤其是抗 Hu抗体 )是国内人群中PNSNS患者较敏感和特异性的血清标志物 ,在神经元的免疫损伤中具有重要作用     

Serotonin and central nervous system syndromes of childhood: A review

A brief review is presented of clinical studies of serotonin (5-hydroxytryptamine) (5-HT) and its metabolites in children with diseases of the central nervous system. Evidence of 5-HT abnormalities in the hyperactive syndromes, primary infantile autism, and childhood schizophrenia is reviewed and compared with the many types of mental retardation with documented serotonin abnormalities. The platelet model system is postulated to explain the relationship of serotonin abnormalities in blood to clinical evidence of brain dysfunction in these varying patient groups. The nonspecificity of serotonin abnormalities in humans is emphasized.     

Central nervous system lymphoma associated with natalizumab

Patients with primary central nervous system lymphoma (PCNSL) after treatment with natalizumab have been considered co-incidental. We report another case of PCNSL in a patient where the explosive onset suggests a causal link.     

Central nervous system germinomas. A review

The germinoma represents a less malignant form of germ cell tumor. Depending on the individual's age, this neoplasm constitutes approximately 0.1% to 3.4% of all intracranial tumors. The embryologic origin remains a mystery; however, current theories implicate an aberration in primordial germ cell migration. Clinical presentation depends on tumor location and may involve endocrine, hypothalamic, visual, and cognitive dysfunction. In evaluating midline intracerebral masses, it is imperative that one be aware of the various radiologic appearances, endocrinologic changes, and chemical markers that help to distinguish germinomas from other neoplasms that appear in the pineal, suprasellar, and periventricular regions. Only through the careful evaluation of all available studies can the physician institute appropriate therapies such as biopsy, radiation, and chemotherapy. This article focuses on the epidemiology, embryology, clinical presentation, means of diagnosis, treatment, and outcome of this rare neoplasm.     

神经元表面抗体相关脑炎脑电图特点分析

目的总结神经元表面抗体相关脑炎临床表现、脑电图和头部MRI特点,探讨脑电图对判断疾病复发或波动的意义,以及与MRI病灶相对应的脑电图特点和各临床病程分期的脑电图特点。方法共23例神经元表面抗体相关脑炎患者,根据临床病程分期分为上升期、极期、下降期和恢复期,记录脑电图背景活动、慢波分布范围、样放电和极度δ刷,分析其与疾病复发或波动的关系、与头部MRI表现的一致性,以及各病程分期的脑电图特点。结果 23例患者中19例为抗N-甲基-D-天冬氨酸(NMDA)受体脑炎、3例为抗富亮氨酸胶质瘤失活基因1抗体相关脑炎、1例为抗γ-氨基丁酸B型受体脑炎。临床症状发生率由高至低依次为精神症状或认知功能障碍、癫发作、意识障碍、言语障碍和运动障碍。发病30.50 d内6例脑电图背景活动为慢波,其中2例疾病复发或波动;5例背景活动为α节律,无一例复发或波动。有极度δ刷与无极度δ刷的抗NMDA受体脑炎患者首次住院时间(Z=-0.785,P=0.433)和疾病复发或波动发生率(Fisher确切概率法:P=0.155)差异均无统计学意义。各病程分期脑电图背景活动与头部MRI表现并不完全匹配。上升期和极期脑电图背景活动多为慢波,且慢波分布范围相对较广泛;自下降期开始,背景活动以α节律为主;恢复期慢波分布范围缩小。结论疾病早期脑电图背景活动可能与疾病复发或波动有关。各病程分期脑电图改变与头部MRI表现的不匹配提示应重视神经元表面抗体相关脑炎患者的神经功能检查。不同病程分期脑电图特点不同。     

Central motor conduction in patients with anti-ganglioside antibody associated neuropathy syndromes and hyperreflexia

OBJECTIVES: Several serum antibodies against gangliosides are diagnostically important, particularly in Guillain-Barré syndrome (GBS), Miller Fisher syndrome (MFS), and multifocal motor neuropathy (MMN). Although hyperreflexia is an atypical symptom in these disorders, it has been found in some patients with GBS, MFS, and MMN. The aim of the study was to determine whether hyperreflexia corresponds to corticospinal tract dysfunction in these patients. METHODS: The study examined central and peripheral motor conduction in patients with hyperreflexia who exhibited acute paralysis (group 1, n=5), acute ataxia and ophthalmoplegia (group 2, n=7), or chronic paralysis with conduction block (group 3, n=2). The clinical symptoms are similar to those in patients with GBS, MFS, and MMN, respectively, and serum anti-ganglioside antibodies were found to be positive in all patients. Using magnetic and electrical stimulation techniques, central and peripheral motor conduction were compared in patients in groups 1, 2, and 3 and patients with GBS (n=7), MFS (n=8), and MMN (n=6). RESULTS: Central motor conduction times (CMCTs) in patients in groups 1, 2, and 3 were significantly delayed compared with those in patients with GBS, MFS, and MMN (p<0.01, p<0.05, p<0.05, respectively), and the delayed CMCTs significantly improved in the recovery periods (p<0.01, p<0.01, p<0.05, respectively). However, motor conduction velocity, compound muscle action potential, and F wave conduction velocity were not significantly different between the patients. CONCLUSION: These findings indicate that corticospinal tract is functionally involved in patients with anti-ganglioside antibody associated neuropathy syndromes and hyperreflexia     

Central nervous system side effects associated with zolpidem treatment

Zolpidem is one of the newer medications developed for the treatment of insomnia. It is an imidazopyridine agent that is an alternative to the typical sedative-hypnotic agents. Zolpidem use is gaining favor because of its efficacy and its side effect profile, which is milder and less problematic than that of the benzodiazepines and barbiturates used to treat insomnia. Still, side effects are not uncommon with zolpidem use. We report a series of cases in which the patients developed delirium, nightmares and hallucinations during treatment with zolpidem. We will review its pharmacology, discuss previous reports of central nervous system side effects, examine the impact of drug interactions with concurrent use of antidepressants, examine gender differences in susceptibility to side effects, and explore the significance of protein binding in producing side effects.     

Central nervous system tuberculosis: imaging manifestations

Tuberculosis of the brain is a disease of pathologic complexity and clinical subtlety-characteristics that manifest in the radiologic presentation of the disease and may make the task of interpreting radiologic data difficult. Much of the difficulty, however, reflects differences between the pathologic processes of tuberculosis and other infective diseases of the brain, and once these differences are appreciated, interpreting radiologic images becomes clearer.     

Central nervous system immunity associated with clinical outcome in acute encephalitis

To search for useful laboratory measures of central nervous system (CNS) immunity that may provide an accurate prognosis or clues regarding treatment choice, cerebrospinal fluid (CSF) samples were obtained from 14 consecutive patients with acute encephalitis during acute as well as convalescent or chronic stages, and then examined for surface antigen expressions by lymphocytes and the presence of antineuronal tissue antibodies as well as the levels of IgG-related parameters and proinflammatory cytokines, including IL-2, IL-6, IFN-gamma, and tumor necrosis factor-alpha (TNF-alpha). Seven patients with aseptic viral meningitis served as nonencephalitic controls. Eight of the 14 acute encephalitis patients recovered fully, and reductions in the percentages of CD4(+)CD29(+) helper inducer T cells and IL-2 receptor-positive CD4(+) cells were associated with early recovery and favorable outcome, respectively, whereas a low percentage of CD4(+)CD26(+) memory T cells during an acute stage was associated with an unfavorable outcome following adjunctive intravenous corticosteroid treatment. Further, three of the four encephalitis patients who exhibited autoantibodies had a poor prognosis. These findings suggest that CNS immunity status has an effect on prognosis, while flow cytometric analyses of CSF CD4(+) helper T cell subsets may serve as effective means of assessment.     

Central nervous system lymphomas and immunodeficiency

Primary lymphomas of the central nervous system (CNS) may sometimes be associated with some immunological abnormalities, including renal or cardiac transplants, some congenital and acquired immunodeficiencies, immunoinflammatory diseases and immunosuppressive treatments. A relatively high incidence of cerebral lymphomas has been particularly noticed in renal or cardiac transplantation patients and in those with acquired immune deficiency syndrome (AIDS), two conditions which are today observed with increasing frequency. The different congenital and acquired immunodeficiencies associated with cerebral lymphomas and the pathogenetic connections between the two conditions are discussed from a large review of the literature.     

中枢神经系统损伤后神经再生的策略

中枢神经系统（central nervous system，CNS）损伤后神经细胞受损、缺失或死亡，常使神经功能严重受损而导致偏瘫、失语、智力障碍或昏迷，甚至死亡。传统的药物治疗及功能性电刺激虽然显示了一定的效果，但是要修复受损的神经环路，重建神经功能，至关重要的是解决神经再生这一难题。由于CNS损伤后复杂的病理生理变化，单一的治疗措施难以获得良好的再生效果，因此，促进CNS损伤后的神经再生需要“多管齐下”。目前，我们认为促进神经再生的策略主要有下述几个方面。[第一段]     

Rat olfactory cells and a central nervous system neuronal subpopulation share a cell surface antigen

Using the monoclonal antibody (Mab) 6B7, a cell surface component found in adult rat central nervous system membrane preparations and on the surfaces of a subpopulation of neurons in cultures of embryonic rat forebrain has been identified. This Mab was derived from mice immunized with a rat forebrain synaptic plasma membrane preparation. High levels of Mab 6B7 binding are observed with membrane preparations from rat forebrain and olfactory bulb but no detectable binding is observed with membranes from the non-neural adult rat tissues heart, kidney, liver, lung and testes. Binding to dorsal root ganglia preparations was 5-fold lower than to forebrain. In immunofluorescence analyses, Mab 6B7 binds to the surface of a significant proportion of neurons in cultures of embryonic day 14 rat forebrain. However, it is absent from GFAP-positive astrocytes, oligodendrocytes, Schwann cells and fibroblastic cells in rat neural cultures. Due to the high levels of binding in olfactory tissue, the distribution of the 6B7 antigen in the olfactory epithelium was characterized in greater detail. In cryostat sections, 6B7 appears to react with a cell population of the basal layer of the adult rat epithelium, but is absent from the cell bodies of the more mature neuronal population which lies higher in the epithelium. This result suggests that within the olfactory epithelium Mab 6B7 may be useful as a marker for the proliferative basal cells which are the neuronal precursors in the epithelium. In summary, the 6B7 antigen may be useful in identifying and analyzing cell subpopulations in both the central nervous system and olfactory epithelium.     

儿童抗MOG抗体及抗NMDAR抗体双阳性中枢神经系统脱髓鞘1例报道并文献复习

目的 探讨缺血性脑卒中(Cerebral ischemic stroke,CIS)患者发生睡眠呼吸紊乱(Sleep-disordered breathing,SDB)的影响因素。方法 纳入2018年2月-2019年4月本院神经内科收治的288例首次发病的CIS患者,于发病后的第7 d左右采用Apnea Link^TM睡眠测试装置整夜连续进行呼吸睡眠检测,发生SDB的患者[呼吸暂停-低通气指数(Apnoea-hypopnoea index,AHI)≥10]纳入研究组,未发生SDB的患者纳入对照组,考察CIS患者中SDB的发生率,采用单因素分析的方法比较发生SDB的可疑危险因素,对于单因素分析有意义的自变量,采用Logistic回归逐步向前法进一步识别SDB的独立影响因素。结果 CIS患者中SDB的发病率为63.1%。多因素Logistic回归分析显示,年龄≥65岁[OR(95%CI)=2.078(1.317～3.28),P=0.002]、脑干病变[OR(95%CI)=2.306(1.418～3.75),P=0.001]、NIHSS得分[OR(95%CI)=2.368(1.34～4.185),P=0.003]、mRS得分[OR(95%CI)=2.033(1.146～3.606),P=0.015]和发生吞咽困难[OR(95%CI)=2.392(1.031～5.545),P=0.042]是脑卒中患者发生SDB的独立危险因素。结论 脑干损伤是脑卒中患者发生SDB的主要危险因素,而SDB加重了急性期缺血性脑卒中患者的神经系统症状,老年缺血性脑卒中患者是SDB的易感人群。对脑干损伤的老年患者,要重视早期监测呼吸睡眠功能,发生SDB迹象时要及时采取干预措施。