运动神经元疾病患者血清和脑脊液中GM1抗体的分布及其意义

目的分析运动神经元疾病（ＭＮＤ）患者血清和脑脊液中神经节苷脂（ＧＭ１）抗体的分布，研究其在ＭＮＤ发病过程中的作用。方法采用ＥＬＩＳＡ方法检测了４５例不同类型的ＭＮＤ患者血清和脑脊液中ＩｇＭＧＭ１、ＩｇＧＧＭ１抗体的水平。结果ＭＮＤ患者血清中ＩｇＭＧＭ１、ＩｇＧＧＭ１抗体均明显高于对照组，其中ＩｇＭＧＭ１抗体在进行性脊肌萎缩症患者中阳性率最高，而ＩｇＧＧＭ１抗体在ＭＮＤ的不同临床类型中差异无显著性意义。ＭＮＤ患者脑脊液中ＩｇＭＧＭ１和ＩｇＧＧＭ１抗体也均明显高于对照组（Ｐ＜０．０１），在ＭＮＤ不同临床类型中差异无显著性意义（Ｐ＞０．０５）。ＭＮＤ患者脑脊液中ＩｇＭＧＭ１和ＩｇＧＧＭ１抗体与血清中ＩｇＭＧＭ１和ＩｇＧＧＭ１抗体均不相关。结论ＭＮＤ患者周围和鞘内均存在对ＧＭ１的免疫反应，并可能参与了ＭＮＤ的发病过程。     

高热惊厥患儿血清及脑脊液 NSE 的测定及其意义

目的：测定高热惊厥患儿血清及脑脊液神经元特异性烯醇化酶（NSE）的表达水平,探讨高热惊厥（FC）患儿惊厥发作后有无并发脑损伤。方法采用ELISA法测定50例单纯性FC患儿,50例复杂性FC患儿及48例对照儿童血清及脑脊液NSE表达水平,并进行统计学分析。结果（1）复杂性 FC组血清 NSE水平（26.31±9.76 ng/mL）高于单纯性 FC组（21.73±8.33 ng/mL ;P＜0.05）及对照组（13.48±7.67 ng/mL ;P＜0.01）,单纯性 FC组血清 NSE水平高于对照组（P＜0.05）;（2）复杂性FC组和单纯性FC组脑脊液NSE水平分别为（19.33±5.83）ng/mL和（17.40±6.11）ng/mL ,均高于对照组的（11.06±5.85）ng/m L分别 P＜0.01,＜0.05,复杂性FC组和单纯性FC组差异无统计学意义（ P＞0.05）。结论高热惊厥患儿惊厥发作后有一定程度的脑损伤发生。     

急性脑血管病患者血清NSE测定及其临床意义

目的 :评价脑血管病患者血清神经元特异性烯醇化酶 (NSE)水平及其临床意义。方法 :用ELISA法对 38例脑梗死及 13例脑出血患者发病后 1、2、3、15d和 2 7例正常对照血清NSE水平进行检测 ,并与脑损害大小及 1个月后ADL评分进行相关分析。结果 :脑血管病患者血清NSE水平明显高于正常对照 ,脑梗死患者血清NSE均值、峰值水平与梗死体积的相关系数分别为 0 77、0 81(P <0 0 1) ,两者与ADL的相关系数分别为 -0 35 (P <0 0 5 )、-0 37(P <0 0 5 ) ;脑出血患者则无此相关性。结论 :血清NSE可以反映脑梗死程度及其近期预后 ,动态检测可以指导重症脑血管病的治疗     

脑梗死患者血液和脑脊液NSE质量浓度测定及其临床意义的相关性研究

目的　观察急性期和恢复期脑梗死患者血液和脑脊液的神经元特异性烯醇化酶 (neuron-specific enolase,NSE)质量浓度变化 ,探讨其与神经功能缺损程度、脑梗死体积、颅内压以及患者年龄等方面的相关性。方法　该实验采用酶联免疫吸附法 (enzyme-linked immunosorbent assay,ELISA) ,检测观察组 (4 6例 )、对照组 (2 5例 )血液及脑脊液 NSE质量浓度 ,并应用 SPSS1 0 .0统计软件包进行统计学分析。结果　急性脑梗死 (acute cere-bralinfarction,ACI)患者血液和脑脊液 NSE质量浓度显著高于恢复期患者和对照组 (P <0 .0 1 ) ;ACI患者脑脊液 NSE质量浓度显著高于血液 (P <0 .0 1 ) ;血液和脑脊液 NSE质量浓度与梗死体积均呈显著正相关 (P <0 .0 1 ) ,与出院时神经功能缺损程度呈显著正相关 (P <0 .0 1 )。结论　脑脊液或血清中的 NSE质量浓度是脑组织破坏后较合适的生化标记物 ,有助于判断脑梗死患者梗死范围、监测病情变化及疗效观察。     

颅脑损伤患者早期血清IL-6和NSE的变化及临床意义

目的探讨颅脑损伤患者早期血清IL-6和NSE的变化及与颅脑损伤程度的关系。方法用双抗体夹心酶标免疫分析法测定60例颅脑损伤患者血清IL-6和NSE的水平。结果60例颅脑损伤患者血清IL-6和NSE水平均有升高,其水平与颅脑损伤的严重程度显著相关(P<0.001),并且颅脑损伤患者血清IL-6和NSE含量之间呈正相关(r=0.417,P<0.005)。结论血清IL-6是评价颅脑损伤早期炎症损伤程度的一项重要的生化指标。     

NSE在手足口病合并脑炎患儿血清和脑脊液中的水平及临床意义

为了解神经元特异性烯醇化酶(NSE)在手足口病合并脑炎患儿血清及脑脊液中的水平,以及对临床诊断与治疗的指导作用,我们对2011-05—2012-04在我院住院的手足口患儿进行NSE检测,现报道如下。1对象与方法1.1一般资料所有病例均为2011-05—2012-04在我院就诊并确诊为手足口病的住院儿童,普通病例30例,合并脑炎40例,分列为普通组和合并脑炎组,普通组男16例,女14例;中位年龄3.5岁;合并脑炎组男22例,女18例;中位年龄2.6岁。所有患儿临床诊断及分期均符合卫生部2010年《手足口病诊疗指南(2010年版)》和肠道病毒71型(EV71)     

脑出血患者血浆NSE的测定及其意义

本文用双抗体夹心酶标记免疫分析法动态检测３０例脑出血患者发病一周内（第１－７天）血浆中神经元特异性烯醇化酶（Ｎｅｕｒｏｎ－ｓｐｅｃｉｆｉｃ ｅｎｏｌａｓｅ，ＮＳＥ）的浓度，结果显示脑出血生期内血浆ＮＳＥ增高，发病一周内的平均ＮＳＥ值及最大ＮＳＥ值均与ＣＴ显示的脑血肿体积呈正相关。高ＮＳＥ的屠 病情重，预后不良；病情轻１、预后良好者，ＮＳＥ仅轻度上升。但ＮＳＥ升高不显著者，并不一定意味着病情轻、预的     

运动神经元病血清抗神经节苷脂抗体的检测及临床意义

本研究用ELISA法初步检测了MND各类型，其它神经系统疾病（OND）及非神经系统免疫性疾病（NID）血清中抗GM1抗体的反应性。发现MND患者与正常对照组抗GM1抗体反应性有显著差异。26例中有8例（38％）MND患者抗GM1抗体滴度明显高于正常对照组，MND患者与OND对照组抗GM1抗体A值有显著差异。在部分NID息者也存在较高滴度的IgM抗GM1抗体。运动神经元病（MND）病因学说尚无统一认识。进一步开展抗CM1抗体的检测、阐明其在运动神经元疾病各亚型中的分布规律及反应性有助于深入研究其发病机制及治疗。     

急性脑梗死患者血清NSE分析

目的探讨急性脑梗死(ACI)患者血清中神经元特异性烯醇化酶(NSE)水平及其与脑梗死面积大小的关系。方法对210例ACI患者按脑梗死面积大小进行分组,采用双抗体夹心ELISA法进行血清NSE水平测定。结果ACI患者各亚组间血清NSE水平有显著性差异(F值=16.2,P0.05),且与梗死灶体积呈显著正相关(r=0.72,P0.05)。结论ACI患者血清NSE水平可反应ACI患者的脑梗死状态,对判断病情和预后有重要价值。     

颅脑损伤患者血清NSE的含量变化及临床意义

目的动态监测颅脑损伤患者血清神经元特异性烯醇化酶(NSE)的含量变化,并探讨NSE与颅脑损伤程度及其预后之间的关系。方法用酶联免疫测定法检测86例颅脑损伤患者的血清NSE含量,并分析其与颅脑损伤严重程度及患者预后的关系。结果颅脑损伤患者血清NSE水平明显高于对照组(P<0.05);重度颅脑损伤患者血清NSE水平又明显高于中、轻度颅脑损伤者(P<0.05);重度颅脑损伤预后不良者的血清NSE水平则明显高于预后良好者(P<0.05)。结论血清NSE水平与脑损害的严重程度呈正相关,可作为评估颅脑损伤预后的重要指标。     

运动神经元病

运动神经元病是一组散发或遗传的神经变性病。主要累及运动神经元,病程进展而死亡。文中就其临床表现和诊断标准、流行病学和遗传学进行综述。     

抗GM1抗体和抗脑磷脂抗体与运动神经元疾病

目的通过分析运动神经元疾病MND）患者血清中抗神经节苷脂GM1抗体和抗脑磷脂抗体，探讨MND的免疫机制。方法用ELISA法测定了22例MND患者血清的抗神经节苷脂GM1抗体和抗脑磷脂抗体。结果MND患者抗GM1抗体阳性7例（32％），抗脑磷脂抗体阳性5例（23％），两种抗体均阳性者2例（9％）；在下运动神经元损害为主的MND（U＋L－MND）患者16例中，7例（44％）抗GM1抗体阳性，5例（32％）抗脑磷脂抗体阳性，而上运动神经元损害的MND（U－MND）患者中二种抗体阳性率均为17％（1例），两者比较亦有显著性差异（P＜0．01）。结论MND可能与自身免疫功能失调有关，尤其在L－MND和U＋L－MND中，自身免疫机制可能参与其疾病的发生和发展。     

IgM-神经节苷脂GM_1抗体在运动神经元病中的初步观察

26例运动神经无病(MND)患者血清中IgM-神经节苷脂GM_1抗体检测的阳性率为65％,明显高于其他神经疾病(OND)组11％及正常人(NC)组。抗体效价在MND组为289.20±183.66,明显高于OND组(97.50±60)及NC组(<50),抗体效价>350时,仅见于MND组,各类MND的GM_1抗体阳性数及效价以下运动神经元病(LMND)最高,肌萎缩侧束硬化(ALS)次之,上运动神经元病(UMND)最低,GM_1抗体效价与病情相关(P<0.01),病情重、效价高。     

Do GM1 antibodies induce demyelination?

We review clinical, neurophysiological, immunological, and experimental data concerning multifocal motor neuropathy (MMN), a newly recognized disorder that mimics MND. It is separated from MND by the presence of multifocal conduction block (CB) demonstrated electrophysiologically, and in some instances by the association of high liters of GM₁ antibodies. The possible immunopathogenetic effect of GM₁ antibodies is discussed. However, 70% of patients with MMNCB do not have elevated titers of GM₁ antibodies, but may respond nevertheless to immunosuppressive treatment. Thus, so far unrecognized antibodies may react against some other epitopes in the paranodal region than those attacked by GM₁ antibodies to cause CB. © 1994 John Wiley & Sons, Inc.     

NSE、MBP对重型脑外伤的临床评估

目的　研究重型脑外伤后神经元特异性烯醇化酶 (neuron specific enolase,NSE)、碱性髓鞘蛋白 (myelinbasic protein,MBP)血清浓度变化 ,以期为临床重型脑外伤后脑损伤监测及预后评估提供直观的定量指标。方法　对 3 0例重型脑外伤住院患者伤后 1 2 h至第 4天进行连续血清 NSE、MBP浓度检测 ,并结合格拉斯哥预后计分 (GOS)及头颅 CT表现进行比较分析。结果　 3 0例重型脑外伤患者伤后 1 2 h血清 NSE、MBP均显著高于正常对照组 ,且与患者预后密切相关。此后 NSE、MBP浓度虽均呈下降趋势 ,但仍高于正常值 ,预后恶劣组伤后每天 NSE浓度均持续高于预后良好组 ,差异有显著性。结论　重型脑外伤后血清标记物 NSE、MBP浓度与患者预后关系密切 ,且伤后 NSE浓度动态变化对继发性脑损害评估亦有重要意义 ,其为临床救治效果及病情转归的判断提供了有效手段。     

Olfactory Disorder in Motor Neuron Disease

In Parkinson's disease and Alzheimer's disease there is profound disorder of olfaction. The extent to which this modality is involved in motor neuron disease (MND) has been studied little. To address this further we assessed olfaction by three methods—a smell identification test (“UPSIT”) in 58 patients and 135 controls; olfactory-evoked response (OEP) to H₂S in 15 patients, and pathological examination of olfactory bulbs obtained from 8 cadavers. It was found that smell identification compared with the controls was slightly worse overall in the MND group as a whole, but only the bulbar patients scored significantly less on the UPSIT. Patients displayed a subtle defect in cheese odor recognition. OEPs were normal in 9 subjects and delayed in 1 subject. The remaining 5 OEPs were unsuccessful. Histopathological studies of olfactory bulbs showed excess lipofuscin deposition in all 8 cases examined, indicating subclinical neuronal damage. Olfactory neurons with a degree of antioxidant defect may be more susceptible to cellular damage than other neuronal groups because of their direct relationship to environmental agents. Overall we found the degree of olfactory dysfunction in MND to be mild and in contrast with the marked changes described by others.     

Childhood-onset multifocal motor neuropathy with IgM antibodies to GM2 and GalNac-GD1a

BackgroundMultifocal motor neuropathy (MMN) is an acquired immune-mediated form of neuropathy characterized by upper and asymmetric limb weakness without sensory loss. The mean age of onset is 40 years (range, 20–70 years), and childhood-onset MMN is extremely rare. In the present report, we discuss a case of childhood-onset MMN in a patient who tested positive for anti-GM2 and anti-GalNac-GD1a immunoglobulin M (IgM) antibodies.Case reportA 12-year-old girl presented with progressive weakness of the upper extremities without sensory loss. Electrophysiological assessments revealed definite conduction blocks in the left median and bilateral radial nerves. She was diagnosed with MMN in accordance with the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria. Serological studies revealed that she tested positive for IgM antibodies to GM2 and GalNac-GD1a. Partial improvements in both muscle weakness and electrophysiological assessments were achieved after 8 months of high-dose intravenous immunoglobulin (IVIg) treatment.ConclusionAlthough childhood-onset MMN is rare, most patients respond to IVIg treatment. This is the first case of childhood-onset MMN in a patient who tested positive for anti-GM2 and anti-GalNac-GD1a IgM antibodies. Although half of the adult patients with MMN test positive for anti-GM1 IgM antibodies, they were not detected in our patient. Comprehensive testing for serum anti-glycolipid antibodies in addition to GM1 may aid in the diagnosis of childhood-onset MMN.     

Presenile Dementia With Motor Neuron Disease –An Additional Case Report–

Abstract: A 51-year-old male has suffered the gradual onset of difficulty with memory, concentration and cognition at age 50. He has progressively developed severe dementia accompanied by muscle wasting and fasciculation prominent in the upper arms and shoulders and the bulbar muscles. EMG and muscle biopsy revealed denervation patterns and a CT-scan showed moderate cerebral atrophy. Cases of presenile dementia with motor neuron disease reported in Japan have been reviewed and the possibility of a new clinico-pathologic entity is discussed.