一家系遗传性运动感觉性神经病的神经电生理检查

本文报告１家３代遗传性运动感觉性神经病（ＨＭＳＮ）１２例患者及１例无症状者的脑干听觉诱发电位（ＢＡＥＰ）、体感诱发电位（ＭＥＰ）、磁刺激运动诱发电位（ＭＥＰ）、视觉诱发电位（ＶＥＰ）及肌电图（ＥＭＣ）和神经传导速度（ＮＣＶ），结果表明：ＢＡＥＰ、ＳＥＰ、ＭＥＰ、ＶＥＰ、ＥＭＧ和ＮＣＶ的异常率分别为９２％、９０％、９２％、７７％、９４％、９５％。     

急性一氧化碳中毒后迟发性脑病的诱发电位、CT和脑电图研究

目的：研究急性一氧化碳中毒后迟发性脑病（ＤＥＡＣＭＰ）患者的诱发电位（ＥＰ）、ＣＴ和脑电图（ＥＥＧ）。方法：对４６例ＤＥＡＣＭＰ患者进行ＥＰ、ＣＴ和ＥＥＧ检查。结果：异常率体感诱发电位（ＳＥＰ）８３％、视觉诱发电位（ＶＥＰ）６３％，脑干听觉诱发电位（ＢＡＥＰ）３０％，ＣＴ７１％，ＥＥＧ１００％。ＳＥＰ中的Ｐ４０、Ｎ５０、Ｐ６０和Ｎ７５峰潜伏期（ＰＬ）比正常对照组显著延长。ＶＥＰ的Ｐ１００ＰＬ较对照组明显延长。ＢＡＥＰ的Ⅰ、Ⅲ和Ⅴ波ＰＬ及Ⅰ—Ⅲ、Ⅲ—Ⅴ和Ⅰ—Ⅴ峰间期（ＩＰＬ）与对照组比较无显著性差异。结论：ＥＰ、ＣＴ和ＥＥＧ结合临床观察，对ＤＥＡＣＭＰ的定位诊断、病情判断与预后评估均有意义。     

脑瘫病人选择性后根切断术的体感诱发电位、神经传导速度检测

报告２０例脑瘫病人选择性后根切断术（ＳＰＲ）治疗前后体感诱发电位（ＳＥＰ）、运动神经传导速度（ＭＣＶ）检测结果。ＳＥＰ较术前潜伏期缩短者在ＥＢ点为６３％、ＦＯ点４１％、Ｎ１点４１％、Ｐ１点４１％、Ｎ２点５０％，神经传导速度亦较术前明显缩短。对ＳＰＲ的机制进行了讨论。     

病毒激活巨噬细胞产生的一氧化氮与细胞毒的关系

目的：确定新城鸡瘟病毒Ｌｏｄｏｔａ系（ＮＤＶ－Ｌ）能否诱导小鼠腹腔巨细胞（ＰＥＭψ）产生一氧化氮（ＮＯ）以及该 小鼠ＰＥＭψ介导的细胞毒作用是否有依赖关系。方法：采用Ｇｒｉｅｓｓ、ＦＡＣＳ分析和噻唑蓝（ＭＴＴ）方法。结果：ＮＣＶ－Ｌ与ＰＥＭψ任用２Ｈ后就可以稳定地吸附在ＰＥＭψ表面,吸附率达８０％左右;收集ＮＣＶ－Ｌ与ＰＥＭψ作用２４Ｈ上清,发现有ＮＯ的释,释放量与阳性对照组（ＢＸＣＧ－ＬＰＳ－Ｐ     

系统性红斑狼疮患者B淋巴细胞EBV-LMP1和ZEBRA的表达研究

目的：探讨ＥＢＶ－ＬＭＰ１和ＺＥＢＲＡ在系统性红斑狼疮患者（ＳＬＥ）的表达情况。方法：间接荧光免疫标记，流式细胞仪检测。结果：ＳＬＥ患者Ｂ淋巴细胞中ＥＢＶ－ＬＭＰ１和ＺＥＢＲＡ的表达显著高于正常对照组（Ｐ＜０．０１）。活动期患者ＣＤ２３＋细胞ＥＢＶ－ＬＭＰ１和ＺＥＢＲＡ的表达率均高于ＣＤ１９＋细胞（Ｐ＜０．０１）。非活动期患者ＣＤ２３＋细胞ＥＢＶ－ＬＭＰ１表达也高于ＣＤ１９＋细胞（Ｐ＜０．０１）。但ＥＢＶ－ＺＥＢＲＡ表达在两亚群间差异没有统计学意义（Ｐ＞０．０５）。结论：朋病毒参与了ＳＬＥ的发病机制，病毒主要以潜伏期状态存在于患者中，病毒复制促进病情发展，检测Ｂ淋巴细胞ＥＢＶ－ＬＭＰ１和ＺＥＢＲＡ的表达率，有助于病情活动指标的判断。     

脂质过氧化在老化大鼠急性胆源性肝细胞线粒体损害中的作用及维生素E的影响

目的：探讨脂质过氧化在老化大鼠胆源性肝细胞线粒体受损中及维生素Ｅ（ＶＥ）的保护作用。结果：老化大鼠非ＶＥ处理组（ＮＶＥＧ）肝细胞线粒体丙二醛（ＭＤＡ）含量明显高于非老化组，超氧化物歧化酶（ＳＯＤ）和谷胱甘肽过氧化物酶（ＧＰＤ）活性明显低于非老化组，过氧化氢酶（ＣＡＴ）无明显变化。１８和２４月龄ＶＥ处理组（ＶＥＧ）ＭＤＡ显著低于ＮＶＥＧ，ＳＯＤ和ＧＰＤ显著高于ＮＶＥＧ，并以１８月龄组为明显。急性梗阻性左肝胆管炎（ＡＯＬＨ）后ＭＤＡ水平明显高于对照组，间接致伤肝叶（ＩＡＬ）较直接致伤肝叶（ＤＡＬ）为明显；ＩＡＬ线粒体ＳＯＤ和ＣＡＴ活性均显著高于对照组，ＤＡＬ显著降低；各叶ＧＰＤ活性均显著低于对照组，ＩＡＬ和ＤＡＬ均无显著差别；以上改变以２４月龄组为明显。各月龄ＶＥＧＡＯＬＨ２４ｈ时ＩＡＬＭＤＡ含量明显低于ＮＶＥＧ；１８月龄ＶＥＧＡＯＬＨ后ＳＯＤ水平均明显高于ＮＶＥＧ；各月龄ＶＥＧ组ＡＯＰＨ后ＧＰＤ和ＣＡＴ活性与ＮＶＥＧ比较均无明显差异。结论：脂质过氧化是老化大鼠胆源性肝细胞线粒体受损的重要机制，ＶＥ具有明显的保护作用。     

31例格林—巴利综合征肌电图分析

３１例格林—巴利综合征肌电图分析安莉，郭晓滨，刘国荣为了进一步探讨肌电图（ＥＭＧ）和神经传导速度对格林一巴利综合征（ＧＢＳ）的应用，对本院神经内科病房收治的ＣＢＳ患者的ＥＭＧ和运动传导速度（ＭＣＶ）资料，加以整理分析。１临床资料３１例均是经过临床确诊...     

虎杖甙对正常大鼠血管平滑肌细胞内游离钙浓度的影响

目的：观察虎杖甙（ＰＤ）对培养的大鼠血管平滑肌细胞（ＶＳＭＣ）内钙离子浓度的变化机理。方法：用Ｆｌｕｏ－３－ＡＭ标记培养的ＶＳＭＣ，在粘附式细胞仪上测定细胞内游离钙的变化。结果：实验结果表明，ＰＤ（００２～２０ｍｍｏｌ／Ｌ）可使大鼠ＶＳＭＣ内游离钙浓度升高，波形变宽，其引起的钙波形态与去甲肾上腺素（ＮＥ）及氯化钾所引起的高尖钙波不同，ＰＤ使ＶＳＭＣ产生一种剂量依赖性的、持续缓慢升高的钙波。ＰＤ引起的细胞内游离钙升高可被ＥＧＴＡ（２ｍｍｏｌ／Ｌ）及维拉帕米（５０μｍｏｌ／Ｌ）明显抑制。结论：以上结果提示ＰＤ既促进ＶＳＭＣ外钙离子进入细胞内，还能诱导细胞内钙离子释放；ＰＤ可能会提高正常ＶＳＭＣ的收缩性，增加正常血管的张力。     

虎杖甙对正常人血管平滑肌细胞内钙和膜电位的调节作用

目的和方法：观察虎杖甙（ＰＤ）对人脐带动脉平滑肌细胞（ＶＳＭＣ）内游离钙、细胞膜电位的变化,以探讨ＰＤ对血管平滑肌的调节机制。用Ｆｌｕｏ－３－ＡＭ、ＤｉＢＡＣ４（３）标记培养的ＶＳＭＣ,在激光共聚焦显微镜上测定细胞内游离钙和膜电位变化。结果：给ＰＤ（０５ｍｍｏｌ／Ｌ）１０ｍｉｎ后,ＶＳＭＣ内游离钙浓度升高５６％±５６％。当ＰＤ加入前用维拉帕米和ＥＧＴＡ预处理后,则游离钙不再升高;ＥＧＴＡ和肝素预处理也抑制ＰＤ的升钙作用,而ＥＧＴＡ和普鲁卡因预处理则使细胞内钙显著升高。ＰＤ还可使ＶＳＭＣ膜电位去极化,加入钠通道阻断剂河豚毒素（２５μｍｏｌ／Ｌ）可完全阻断ＰＤ的去极化作用：加甲氰咪胍、维拉帕米、优降糖和利及丁预处理不能阻断ＰＤ去极化作用。结论：：ＰＤ可通过细胞外钙内流来增加细胞内游离钙浓度,并促进细胞外钠离子内流而导致细胞去极化     

遗传性运动感觉性神经病合并肥厚性心肌病的3D-TCD、rCBF、BAEP和ECG的研究

目的了解遗传性运动感觉性神经病（ＨＭＳＮ）合并肥厚性心肌病（ＨＣＭ）的经颅多普勒（ＴＣＤ）、局部脑血流量（ｒＣＢＦ）、脑干听觉诱发电位（ＢＡＥＰ）和心电图（ＥＣＧ）的变化。方法对一家三代ＨＭＳＮ合并ＨＣＭ１２例患者和１例无症状者常规进行这四项检查。结果ＴＣＤ、ｒＣＢＦ、ＢＡＥＰ和ＥＣＧ的异常率分别为８５％、７６％、９２％和９２％。结论绝大多数ＨＭＳＮ合并ＨＣＭ患者的ＴＣＤ、ｒＣＢＦ、ＢＡＥＰ和ＥＣＧ均异常。     

肌电图在周围神经损伤诊断中的应用价值

目的：探讨对周围神经损伤的患者进行肌电图（EMG）和运动神经传导速度（MCV）检测的意义。方法：对131例患者损伤神经支配的188块肌肉和166条受损神经进行EMG和MCV检测，并以100例正常对照组进行比较。结果：损伤神经支配的188块肌肉EMG结果均为神经原性损伤，166条受损神经MCV与对照组相比较均有不同程度的减慢以至消失，其中周围神经完全断裂者占21．7％，严重损伤者占21．1％，中度损伤者占24．7％，轻度损伤者占32．5％。结论：对周围神经损伤的定位和损伤程度的诊断，EMG和MCV是可靠和有效的方法。     

Assessment of serum-mediated neurotoxicity in Navajo neuropathy

Navajo neuropathy is a unique sensorimotor neuropathy which is geographically restricted to Navajo children living on the Navajo Reservation. Affected patients present with weakness, loss of sensation in extremities, corneal ulcerations, and a high incidence of childhood infections. Metabolic complications, such as severe liver disease, may further contribute to peripheral nerve injury in affected patients. In this study, serum-mediated injury to rat peripheral nerve was critically assessed. Serum samples from affected Navajo patients were tested in vivo for effects on peripheral nerve function. Injection of serum from affected Navajo patients into rat sciatic nerve produced a modest slowing of nerve conduction velocity without effecting evoked-compound muscle action potential (CMAP) amplitudes. By comparison, injection of serum from patients with MGUS neuropathy, an immune-mediated disorder, diminished evoked-CMAP amplitudes by approximately 70%. Navajo neuropathy sera had no effect in vitro on the neurite outgrowth of developing dorsal root ganglia neurons. The results argue against serum-mediated toxic injury to peripheral nerves in Navajo neuropathy.     

Confocal imaging reveals three-dimensional fine structure difference between ventral and dorsal nerve roots

Peripheral nerve injury repair is one of the most challenging problems in neurosurgery, partially due to lack of knowledge of three-dimensional (3-D) fine structure and organization of peripheral nerves. In this paper, we explored the structures of nerve fibers in ventral and dorsal nerves with a laser scanning confocal microscopy. Thick tissue staining results suggested that nerve fibers have a different 3-D structure in ventral and dorsal nerves, and reconstruction from serial sectioning images showed that in ventral nerves the nerve fibers travel in a winding form, while in dorsal nerves, the nerve fibers form in a parallel cable pattern. These structural differences could help surgeons to differentiate ventral and dorsal nerves in peripheral nerve injury repair, and also facilitate scientists to get a deeper understanding about nerve fiber organization.     

Age-dependent VEGF expression and intraneural neovascularization during regeneration of peripheral nerves

The physiologic ability of peripheral nerves to regenerate after injury is impaired with aging. However, the mechanisms responsible for this phenomenon are still incompletely characterized. In this study, we investigated whether aging influences the intraneural angiogenic response that occurs after injury and during regeneration of peripheral nerves. We performed a crush injury of the sciatic nerve in old and senescence accelerated mice and found that the peripheral nerves of these animals are unable to locally upregulate vascular endothelial growth factor (VEGF), a prototypical angiogenic cytokine, after injury and have substantial deficits in mounting an appropriate intraneural angiogenic response during nerve regeneration.

Our findings provide new evidence of possible interdependent relationships between aging, VEGF, angiogenesis, and nerve regeneration and suggest that vascular abnormalities might play a role in aging-associated neurological dysfunction, with potentially important fundamental and clinical implications.     

Lectin histochemistry applied to human nerves

Histologic sections of normal and pathologically altered human peripheral nerves were stained with a battery of 20 fluorescein isothiocyanate-labeled lectins to determine whether these histochemical reagents could be used to identify peripheral nerve injury. Eight plant lectins--from Canavalia ensiformis, Lens culinaris, Triticum vulgare (wheat germ), types E4 and L4 from Phaseolus vulgaris, types I and II from Ricinus communis, and Wistaria floribunda--were found to bind to normal and pathologically altered nerves. Only two lectins, from Helix pomatia and Maclura pomifera, were found to bind selectively to pathologically altered nerves. The changes recognized by lectin histochemistry were not pathognomonic of any specific type of nerve injury. This study provides baseline data on the reaction pattern of human peripheral nerve with a series of lectins and shows that lectin histochemistry could provide means for the study of peripheral nerve pathology.     

Quantitative analysis of the anatomy of the epineurium of the canine recurrent laryngeal nerve

The purpose of this investigation was to determine the amount of epineurium surrounding the recurrent laryngeal nerve (RLN) compared with a limb nerve, that to flexor hallicus longus (NFHL). Nerve samples were obtained from 10 adult dogs and studied using scanning electron microscopy and light microscopy to measure the relative proportion of epineurium and the relative proportions of adipose and collagenous tissue comprising the epineurium in both nerves. Significantly greater relative epineurial cross-sectional areas and adipose content were found in the RLN than in the NFHL. Based on observations on noncranial peripheral nerves, the findings indicate that the RLN is better protected against deformational forces associated with compression than stretching forces. The RLN may not be structured well for successful reinnervation after injury. The patterns observed for adipose tissue in RLN epineurial tissue appeared unique compared with those previously reported in peripheral nerves. The primary role associated with adipose tissue is to 'package' the nerve for protection. The RLN is considered to be a vital nerve in the body, as are other cranial nerves. The large proportions of adipose tissue in the epineurium may relate to the importance of protecting this nerve from injury.     

Studies of vasoactive intestinal polypeptide expression in injured peripheral neurons using capsaicin, sympathectomy and mf mutant rats

The increased expression of vasoactive intestinal polypeptide (VIP) in injured peripheral neurons was studied. In contrast to substance P, there was a marked increase, and maintained fast axonal transport, of VIP in rat sciatic nerve after peripheral axotomy. Local capsaicin application to the nerve trunk failed to inhibit the injury-induced VIP increase, and capsaicin even increased VIP levels when applied locally to uninjured nerves. Pharmacological sympathectomy showed that some of the peripheral VIP increase may occur in post-ganglionic sympathetic fibres. The VIP increase after injury appeared unaffected in the mf mutant rat, in spite of its loss of lumbar dorsal root ganglion cells. VIP-staining fibres in the epi- and peri-neurium and perivascular plexuses of sciatic nerve showed an increase in number in parallel with the changes of the nerve VIP content. These findings suggest that sensory and sympathetic nerve fibres expressing VIP after injury play a role in the regulation of blood flow to nerves, and in the pathophysiological processes in nerve and dorsal spinal cord which follow peripheral nerve injury.     

改良的下肢SLSEP检测方法对糖尿病近端神经病的诊断意义

目的：探讨改良的短潜伏期体感诱发电位（SLSEP）（改变记录点）的检测方法对糖尿病性近端神经病的诊断意义。方法：排除脑卒中、腰颈椎病及其它疾病引起的神经肌肉疾病,肌电图检查排除有神经病变体征同时常规神经传导检测异常的糖尿病（DM）患者,将符合标准的61例2型DM患者分为3组：有神经病体征而常规神经传导检测正常（Ⅰ组）20例,无神经病体征且常规神经传导检测正常（Ⅱ组）21例和无神经病体征而常规神经传导检测异常（Ⅲ组）20例,与30例正常人均行改良的SLSEP测定。结果：改良的胫神经SLSEP反映下肢感觉神经近端功能的参数总异常率以DM有神经病变症状肢体亚组（Ⅰ组）（n=27）所占的比例最高（85％,23／27）,SLSEP反映神经近端功能的N13、N24峰的感觉神经传导速度（SCV）和N13N24、N9-N24峰间SCV,与对照组比较差异均有显著意义（P〈0．01）;DMⅡ组虽然无神经病变体征并且神经传导测定无异常,但与对照组比较,N13-N24峰间SCV异常肢体率却有显著差异（P〈0．01）。DMⅢ组SLSEP各参数异常率普遍较高（20％～72．5％）,但以反映神经远端功能的N9峰SCV异常率最高。结论：改良的SLSEP检测是对常规SCV检测的一种补充,可以提供感觉纤维近端信息,对有周围神经损害症状而常规SCV测定正常的DM患者其诊断意义尤为显著。SLSEP还有利于发现DM患者亚临床症状。     

Lack of tocopherol in peripheral nerves of vitamin E-deficient patients with peripheral neuropathy

Vitamin E deficiency is often associated with symptoms of a peripheral neuropathy. To evaluate whether vitamin E deficiency affects the vitamin E content of the peripheral nervous system, we measured the alpha-tocopherol content in biopsy specimens of sural nerve and adipose tissue from 5 patients with symptomatic vitamin E deficiency (2 with homozygous hypobetalipoproteinemia and 3 with familial isolated vitamin E deficiency) and 34 control patients with neurologic diseases without vitamin E deficiency. A significant reduction in tissue tocopherol content was present in the vitamin E-deficient patients, as compared with the controls, both in sural nerves (1.8 +/- 1.2 vs. 20 +/- 16 ng per microgram of cholesterol [P less than 0.001], or 7.7 +/- 5.4 vs. 64 +/- 44 ng per milligram of wet weight [P less than 0.01]) and in adipose tissue (46 +/- 43 vs. 222 +/- 111 ng per milligram of triglyceride [P less than 0.001]). Levels of tocopherol in adipose tissue were significantly correlated (P less than 0.001) with levels in peripheral nerves. The low tocopherol content of the nerves preceded histologic degeneration in three vitamin E-deficient patients, suggesting that the nerve injury resulted from the low nerve tocopherol content.     

皮神经感觉传导速度和波幅检测对糖尿病周围神经病的诊断作用

目的：探讨皮神经感觉传导速度（SCV）,波幅（Amp)检测对糖尿病患者周围神经远端受损的早期诊断价值。方法：受试者为糖悄病患者组和正常对照组各25例,对前臂外侧皮神经和腓肠神经共100条,就其感觉传导速度（SVC）及其诱发的波幅（Amp)进行分析,并与胫神经,正中神经进行比较,结果：混合神经的异常率明显低于皮神经,尤以无周围神经症状的糖尿病者更加明显（P＜0．01）,结论：皮神经的SCV,Amp检测,对糖尿病周围神经远端受损的早期诊断可靠,灵敏度高。