奥氮平致低血钾性麻痹1例

1病例 患者男性,47岁。因交替情绪高涨、低落近10年,复发情绪低落半年,诊断为双相情感障碍（抑郁发作）。所查血、尿常规,肝、肾功能,血脂、血糖及血钾均在正常范围,糖化血红蛋白6．2％（正常4．3％～6．1％）。既往体健,否认糖尿病史。给予奥氮平（商品名：再普乐）5—10mg／d治疗,     

隔日顿服强的松治疗重症肌无力疗效及安全性观察

目的 探讨隔日顿服强的松治疗重症肌无力（MG）患者的临床疗效及安全性。方法 对116例MG患者的临床及副作用表现进行跟踪观察。结果 初诊MG患者隔日顿服渐增法，显效率91．4％；MG危象患者，危象控制后强的松渐减法，均取得成功。未发现肌无力加重现象，副作用发生率为16．4％。结论 强的松可作为治疗MG的第一线药物，采用隔日顿服法临床疗效明显，避免初期肌无力加重现象，减少副作用发生。     

甲基泼尼松龙冲击疗法在神经免疫性疾病中的应用

多发性硬化（MS）、重症肌无力（MG）、格林巴利综合征（GBS）及急性脊髓炎均是神经内科常见的自身免疫性疾病，过去用常规剂量类固醇疗法见效慢、用药时间长、副作用大、且疗效大多不能令人满意。近6年来我们借鉴国外经验采用大剂量甲基泼尼松龙冲击疗法（MPPT）治疗神经免疫性疾病86例，取得了良好的临床效果，大大减少了患者的死亡率及致残率。现将观察结果报道如下。1 临床资料1.1 病例选择 本组病例均系我科住院确诊患者，随机分为（1）MPPT治疗组：86例，男41例，女45例，年龄2～53岁，平均28岁，病程3天～7年。包括MS 16例（…     

中药复方扶正强筋片治疗重症肌无力的前瞻性研究

目的 探讨复方中药制剂扶正强筋片治疗重症肌无力（MG）的有效性、安全性及使用时机。方法 收集2004-02—2004-04确诊的164例MG患者，随机分为A组（泼尼松＋溴吡斯的明）、B组（泼尼松＋溴吡斯的明＋扶正强筋片）和C组（扶正强筋片），评估扶正强筋片的起效时间、疗效、使用时机及其不良反应。结果 单用扶正强筋片组于治疗后1个月左右起效，至观察终点有效率为46．9％；扶正强筋片在泼尼松减量或停药时能明显减少患者复发率，B组（10．5％）和C组（6．67％）复发率明显低于A组（31．3％）（P〈0．01），并能减少上呼吸道感染的发生率，且无明显不良反应。OssermanⅠ型患者疗效与性别、年龄及伴发病无明显相关性。结论 单用扶正强筋片治疗MG疗效不肯定，但与泼尼松联用能减少不良反应发生，在泼尼松减量或者停药时可有效预防症状反跳。     

慢性锂中毒临床分析

目的:探讨血锂浓度≥1.0mmol/L的慢性锂中毒患者血锂浓度、中毒症状与治疗之间的关系。方法:查阅150例血锂浓度≥1.0mmol/L的慢性锂中毒患者的病历记录,采集人口学资料及临床特征。结果:高剂量、高龄、女性、合并苯二氮艹卓类药物者血锂浓度较高。中毒症状与血锂浓度密切相关,血锂浓度越高,越容易中毒。血液透析对锂的排除率高于常规治疗。结论:长期服用锂盐的患者应监测血锂浓度,警惕危险因素,临床医生需密切观察中毒症状。     

连续应用7．5％高渗盐水治疗重型颅脑损伤的临床观察

目的 探讨7．5％高渗盐水治疗重型颅脑损伤的效果及副作用。方法 将50例颅脑损伤患者随机分为高渗盐水治疗组（HTS组，26例）和甘露醇治疗组（M组，24例）。HTS组静脉滴注7．5％高渗盐水2ml／kg，M组静脉滴注甘露醇250ml，均为q8h，连续7d。用药前及用药后第1、3、7天测患者血电解质、渗透压和肾功能，用药前及用药后2周对患者进行GCS评分。结果 与用药前相比，用药后6h两组患者的平均动脉压、心率、呼吸均无统计学差异（P〉0．05），而HTS组患者中心静脉压在用药后明显升高（P〈0．05）。用药后第1、3、7天HTS组患者血K^＋、Na^＋、Cl^-、尿素氮、肌酐及血浆渗透压均无显著改变；M组血K^＋、Na^＋、Cl^-、肌酐及血浆渗透压无显著改变（P〉0．05），但用药后第7天尿素氮值较同期HTS组明显升高（P〈0．05）。用药2周后，两组患者GCS评分均有明显改善（P〈0．05）。结论 7．5％高渗盐水降低颅脑损伤引起的高颅内压是安全、有效的，连续使用效果好、副作用少。     

综合降低血氨浓度治疗乙肝肝硬化引起的肝性脑病

目的通过综合降低血氨浓度,观察治疗乙肝肝硬化引起的肝性脑病的临床疗效。方法将56例乙肝肝硬化出现肝性脑病患者随机分为综合降低血氨治疗组（包括口服乳果糖、醋酸灌肠、乙酰谷酰胺静脉注射,n=30）和乙酰谷酰胺治疗组（n=28）,1周治疗后观察临床疗效。结果综合治疗组显效21例（70．0％）,有效9例（30．0％）,无效0例,无死亡,总有效率100％;而乙酰谷酰胺治疗组显效8例（28．6％）,有效8例（28．6％）,无效7例（25．0％）,死亡5例（17．8％）,总有效率为57．1％,2组总有效率比较差异有统计学意义（P〈0．001）。综合治疗组死亡率和血氨浓度也显著降低（P〈0．05）。结论综合降低血氨浓度是控制乙肝肝硬化患者肝性脑病的有效方法,可显著降低肝性脑病的发生,降低患者死亡。     

珍氯片与氯丙嗪治疗精神分裂症的对照研究

目的：评价珍氯片治疗精神分裂症的疗效和安全性，方法：160例精神分裂症病人分为珍氯片嗪治疗二组，疗程6周用BPRS，GI和TESS评价疗效和毒副作用，结果：珍氯片组总有效率76．3％，氯丙嗪组为83．8％（P＞0．05）。心动过速、口干、便秘等副作用珍氯片组发生率低于氯丙嗪组（P＜0．05）。结论：珍氯片治疗精神分裂症疗效与氯丙嗪相似，但服用剂量相对较低，副作用较少。     

右旋佐匹克隆治疗失眠症随机双盲对照研究

目的：评价右旋佐匹克隆治疗失眠症患者的疗效和安全性。方法：257例失眠症患者随机分为研究组（131例）和对照组（126例）。研究组给予右旋佐匹克隆和佐匹克隆模拟剂,对照组给予佐匹克隆及右旋佐匹克隆模拟剂,治疗2周。采用睡眠障碍量表（SDRS）评定疗效。基线和终点时进行血生化、血常规、尿常规以及心电图检查,以评价安全性。结果：两组的SDRS评分在治疗2周末较基线均明显下降（P〈0．01）,而两组间比较差异无统计学意义（P〉0．05）。治疗2周,研究组和对照组在有效率（71．0％,69．8％）方面相当（P〉0．05）,1周后随访,两组的症状反跳率（2．0％,3．1％）无显著差异。研究中未出现严重不良事件,两组间不良事件的总发生率差异无统计学意义（P〉0．05）。结论：右旋佐匹克隆治疗失眠安全有效。     

急性苯妥英钠中毒23例临床及血药浓度监测

报告23例癫痫患者急性苯妥英钠中毒的临床表现，经停服苯妥英纳、应用脱水利尿剂加速药物排泄及对症处理，多数病例于48h－96h中毒症状消失。血药浓度监测显示，中毒时血苯妥英钠浓度在25．3μg/ml－38．2μg/ml（平均31．2μg/ml）。停药后多数病例于48h－96h血苯妥美钠降至治疗范围（10μg/ml-20μg/ml）。血药浓度降至治疗范围者，临床中毒症状消失。于血苯妥英销降至正常范围后重新给予治疗剂量的苯妥英钠（4mg－6mg·kg-1·d-1），观察2周，未见中毒症状再现及癫痫复发。     

Gender prevalence in childhood multiple sclerosis and myasthenia gravis

Multiple sclerosis and myasthenia gravis are the most common autoimmune diseases affecting the nervous system, with generally a female predominance in adults. To determine the gender distribution in childhood, we reviewed 28 patients with definite multiple sclerosis and 44 patients with myasthenia gravis with onset before 16 years. In myasthenia gravis, a significant female preponderance was observed only with onset after 10 years of age; earlier disease showed no gender difference. Multiple sclerosis tended to affect boys more than girls before age 10 years and both sexes equally after this age. Gender prevalence in childhood multiple sclerosis and myasthenia gravis is different than in adult series; these results may provide insight into pathogenesis or clinical approach.     

B cells as therapeutic targets in autoimmune neurological disorders

B cells have a fundamental role in the pathogenesis of various autoimmune neurological disorders, not only as precursors of antibody-producing cells, but also as important regulators of the T-cell activation process through their participation in antigen presentation, cytokine production, and formation of ectopic germinal centers in the intermeningeal spaces. Two B-cell trophic factors-BAFF (B-cell-activating factor) and APRIL (a proliferation-inducing ligand)-and their receptors are strongly upregulated in many immunological disorders of the CNS and PNS, and these molecules contribute to clonal expansion of B cells in situ. The availability of monoclonal antibodies or fusion proteins against B-cell surface molecules and trophic factors provides a rational approach to the treatment of autoimmune neurological diseases. This article reviews the role of B cells in autoimmune neurological disorders and summarizes the experience to date with rituximab, a B-cell-depleting monoclonal antibody against CD20, for the treatment of relapsing-remitting multiple sclerosis, autoimmune neuropathies, neuromyelitis optica, paraneoplastic neurological disorders, myasthenia gravis, and inflammatory myopathies. It is expected that ongoing controlled trials will establish the efficacy and long-term safety profile of anti-B-cell agents in several autoimmune neurological disorders, as well as exploring the possibility of a safe and synergistic effect with other immunosuppressants or immunomodulators.     

Plasma exchange in neuroimmunological disorders: Part 1: Rationale and treatment of inflammatory central nervous system disorders

Plasma exchange is a well-established therapeutic procedure commonly used in many neurological disorders of autoimmune etiology. It is thought that the beneficial effects of plasma exchange occur through the elimination of pathognomonic inflammatory mediators, including autoantibodies, complement components, and cytokines. In various neurological disorders, randomized controlled studies have demonstrated the efficacy of plasma exchange (eg, in Guillain-Barré syndrome and other forms of immune neuropathies). Although widely used, the potential benefit of plasma exchange in the treatment of multiple sclerosis, myasthenia gravis, and Lambert-Eaton syndrome is less clear.     

Coexistence of multiple sclerosis and myasthenia gravis

A young female is reported in whom multiple sclerosis and myasthenia gravis coexisted. The coexistence of multiple sclerosis with other autoimmune diseases and associations with histocompatibility antigens are discussed.     

Heat-shock proteins in clinical neurology

Heat-shock proteins (HSPs) are antigen-presenting protein-aggregation-preventing chaperones, induced by cellular stress in eukaryotic cells. In this review, we focus on recent HSP advances in neurological disorders. In myasthenia gravis, patients responding to immunosuppressive therapy have reduced serum HSP-71 antibodies. Generalized and ocular myasthenia gravis patients have elevated serum HSP-70 antibodies, indicating common pathogenic mechanisms. In Guillain-Barré syndrome, HSP-70 antibodies are elevated in serum and cerebrospinal fluid, and serum levels are higher than in myasthenia gravis and multiple sclerosis. In multiple sclerosis, serum HSP-27 antibodies are elevated during relapses providing disease activation marker, while α,β-crystallin expression in brain lesions indicates remission phase initiation. In acute stroke, serum HSP-27 antibodies are elevated irrespective of stroke type and duration. In epilepsy, HSP-27 is induced in patients' astrocytes and cerebral blood vessel walls, and α,β-crystallin is expressed in epileptic foci. In neurodegenerative disorders such as Alzheimer dementia and Parkinson's disease, HSPs are upregulated in brain tissue, and α,β-crystallin modulates superoxide dismutase-1 (SOD-1) tissue accumulation in familial amyotrophic lateral sclerosis. HSPs play an important role in antigen-presentation and tolerance development. Antibody-mediated interference with their function alters immune responses causing neuropathology. The role of HSPs in clinical neurology should be the subject of future investigation.     

Low-titer antibodies reactive with HTLV-I gag p19 in patients with chronic myeloneuropathy

To elucidate the possible association of human T-lymphotropic virus type I (HTLV-I) and chronic neurological diseases, 156 serum samples from patients with various neurological diseases, including multiple sclerosis, chronic progressive myelopathy, chronic inflammatory polyradiculoneuropathy, myasthenia gravis, polymyositis, motor neuron disease, and tension headache, and healthy control subjects were examined for IgG antibodies to HTLV-I by three independent techniques--gelatin particle agglutination test, enzyme-linked immunosorbent assay, and Western blot assay. Specificity of antibodies was assessed by homologous competitive inhibition on Western blot assay. Six patients (3 with chronic progressive myelopathy, 1 with chronic inflammatory polyradiculoneuropathy, 1 with motor neuron disease, and 1 with tension headache) had high-titer HTLV-I antibodies. Twelve patients (5 with multiple sclerosis, 1 with chronic progressive myelopathy, 2 with chronic inflammatory polyradiculoneuropathy, 2 with myasthenia gravis, and 2 with motor neuron disease) had low-titer HTLV-I antibodies that reacted with a single gag protein, p19 or p24, on Western blot assay. In 4 (2 with multiple sclerosis, 1 with chronic progressive myelopathy, and 1 with chronic inflammatory polyradiculoneuropathy) of these 12, the antibodies that were all directed to p19 were determined to be specific by homologous competitive inhibition. In the remaining 8 patients (3 with multiple sclerosis, 1 with chronic inflammatory polyradiculoneuropathy, 2 with myasthenia gravis, and 2 with motor neuron disease), restricted reactions against p19 or p24 were considered to be nonspecific because they were not inhibited by homologous competitive inhibition. The results suggest that in some patients chronic myeloneuropathy diagnosed as chronic progressive multiple sclerosis, chronic progressive myelopathy, and chronic inflammatory polyradiculoneuropathy may be associated with HTLV-I or related retroviruses.     

Anti-myelin-associated glycoprotein antibody in sera from patients with demyelinating diseases

An enzyme-linked immunosorbent assay (ELISA) was developed for quantitating anti-myelin-associated glycoprotein (MAG) IgM antibody in human sera. Absorbance values of anti-MAG antibody were higher than 0.2 at 1:80 of serum dilution in sera from some patients with demyelinating diseases of the central or peripheral nervous systems including multiple sclerosis, subacute sclerosing panencephalitis, Guillain-Barré syndrome, chronic relapsing polyradiculoneuritis and carcinomatous polyneuropathy and also some patients with autoimmune diseases such as collagen diseases and myasthenia gravis. However, absorbance values of anti-MAG antibody in sera from control individuals and patients with some other neurological diseases were less than 0.2 and considered as negative. Because of the reported existence of a cross antigenicity between MAG and lymphocyte, and especially natural killer cells, the possibility of the functional importance of anti-MAG antibody on cellular immunity is discussed with particular reference to the demyelinating diseases.     

Elevations of hemopexin levels in neuromuscular disease.

Hemopexin, a serum glycoprotein that binds free heme and transports it to hepatic parenchymal cells, has been measured by radial immunodiffusion. We have confirmed elevation of serum hemopexin concentration in Duchenne's muscular dystrophy patients and carries, and demonstrated elevations in dermatomyositis/polymyositis and myasthenia gravis, but not in amyotrophic lateral sclerosis. In monkeys, elevations of hemopexin levels were specifically induced by hematin injections, muscle-crush, or myoglobin injections. Myoglobin leakage is the likely explanation of hemopexin level elevation in Duchenne's dystrophy patients and carriers and in dermatomyositis/polymyositis. In myasthenia gravis there might be a slight myoglobin leakage not heretofore suspected; or, the elevation of hemopexin levels might be a new reflection of a dysimmune state in myasthenia gravis, and perhaps as such is a further incrementing factor in dermatomyositis/polymyositis. Hemopexin, presumably as a longer-phase reactant, is sometimes an index of neuromuscular disease when other data are negative or equivocal.     

Recurrent transverse myelitis, myasthenia gravis, and autoantibodies.

A 45-year-old man with a longstanding diagnosis of myasthenia gravis presented with four episodes of transverse myelitis in 5 years. Each episode improved after treatment with steroids. Laboratory studies revealed no evidence of multiple sclerosis or a structural spinal lesion. He had antinuclear and anti-DNA antibodies and the HLA-A1, B8, DR3 haplotype known to be associated with certain autoimmune diseases. We propose an autoimmune cause for the recurrent episodes of myelitis.     

Cold reactive antilymphocyte antibodies in neurological diseases.

Cold reactive (15 degrees C) antilymphocyte antibodies were detected in the sera of 33% of patients with multiple sclerosis, 50% with Guillain-Barré syndrome, 42% with myasthenia gravis, and 38% with polymyositis. We did not detect such antibodies against autologous cells in multiple sclerosis. In multiple sclerosis there was no correlation between the presence of antilymphocytic antibodies and disease activity or duration. In patients with multiple sclerosis, myasthenia gravis, and polymyositis there was no correlation between the presence of cold reactive antilymphocyte antibodies and abnormalities of T or B cell levels.