小儿阴部神经体感诱发电位、骶反射潜伏期正常值检测及影响因素评价

目的建立儿童阴部神经体感诱发电位、骶反射潜伏期正常值并对其影响因素进行评价。方法无下尿路、神经系统和外生殖器疾病的正常儿童共192例,男127例,女65例,年龄1~15岁,平均(7.75±4.96)岁,将受检儿童按年龄大小分成幼儿期、学龄前期、学龄期、青春前期和青春期五组,分别测定阴部神经体感诱发电位和骶反射潜伏期。结果所有检测对象均记录有阴部神经体感头皮诱发电位和骶反射,但在6岁以上77例男性儿童中仅41例(53%)可显示脊髓电位。不同年龄组中阴部神经头皮诱发电位潜伏期和中枢传导时间统计学有差异(P<0.05),年龄、身高与阴部神经体感头皮电位及中枢传导时间有回归关系,统计学差异有显著性意义(P<0.001)。结论儿童骶反射、阴部神经体感诱发头皮电位易检测,脊髓电位可在6岁以上部分男性儿童中检测;年龄和身高对阴部神经中枢段传导有明显影响,年龄是主要影响因素。本研究检测的正常值及其影响因素评价,可供临床借鉴。     

经皮阴部神经电刺激术治疗儿童原发性夜间遗尿症

目的探讨经皮阴部神经低频电刺激术治疗儿童原发性夜间遗尿症的临床疗效。方法原发性遗尿病24例,其中男17例,女7例,年龄6~16岁,每晚遗尿1~3次不等;记录排尿日记、问卷调查表并进行评分;以低频电流经皮刺激阴部神经,疗程1个月,并检测阴部神经体感诱发电位客观评价其临床疗效。结果24例中,22例治疗有效,其中自行觉醒排尿10例,觉醒功能有改善12例,平均遗尿次数由9.23±4.47次/周减少为2.32±1.07次/周(P<0.001),临床评分由18.50±2.79下降为2.83±2.47(P<0.001),治疗后阴部神经体感诱发电位潜伏期明显缩短(P<0.05)。结论经皮阴部神经低频电刺激治疗儿童原发性遗尿症是一种安全、有效的治疗方法。     

Electrophysiological study of pelvic floor musculature of tethered cord syndrome

目的 利用神经电生理检测技术分析脊髓栓系综合征(tethered cord syndrome,TCS)患儿盆底神经和肌肉功能改变的特点,评价TCS患儿盆底神经肌肉损伤的程度.方法 2008年5月至2010年12月对88例TCS患儿组和26例正常患儿行双侧肛提肌、耻骨直肠肌和肛门外括约肌肌电图检查,以及阴部神经运动传导和阴部神经体感诱发电位(PN-SSEP)检查.结果 正常对照组患儿肛提肌、肛门外括约肌和耻骨直肠肌在静息状态时均可见持续收缩动作电位,大力收缩动作电位密集,呈干扰相.患儿组盆底肌肌电图(pelvic floor muscles electromyographic,PFM-EMG)表现为不同程度的神经源性损害,甚至呈现完全失神经支配,肛提肌较肛门外括约肌病变严重.患儿组阴部神经运动传导波幅降低,潜伏期延长,其中15例患儿出现双侧不对称.阴部神经体感诱发电位潜伏期延长,甚至波形消失.结论 盆底神经电生理学检测对了解TCS盆底神经肌肉损害程度,评价大小便功能,估计预后具有重要意义.     

脊髓栓系综合征电生理检测特点及临床意义

利用电生理检测技术,评价脊髓栓系综合征患儿神经功能改变。方法对ＴＣＳ患儿组及对照组行胫后神经体感诱发电位、胫后神经及腓总神经运动神经传导速度检测。结果ＴＣＳ患儿组ＰＴＮ－ＳＥＰ检测出现皮层电位潜伏期延长,波幅下降甚至消失,部分马尾电位波幅消失;ＭＣＶ轻、中度减慢、动作电位波幅下降,甚至出现传导阻滞,病变随病情而加重。     

神经电生理检查在儿童药物注射性坐骨神经伤中的诊断价值

对５０例儿童坐骨神经药物注射伤损伤部位以远的神经传导速度和波幅及体感诱发电位的结果分析并进行了相关的实验研究。在检查的５０例腓神经中有２７例引不出动作电位，４１例胫神经中有６例引不出动作电位。余２３条腓神经、３５条胫神经传导速度和波幅患侧均较健侧明显减慢和降低。同时对２３例患儿行体感诱发电位检查，结果表明患侧潜伏期较健侧明显延长。实验结果证实在损伤早期动作电位波幅的改变比传导速度的改变更明显，更有诊断价值。     

Erb 点刺激在儿童吉兰-巴雷综合征早期诊断中的作用

目的 探讨Erb 点刺激在儿童吉兰-巴雷综合征(GBS)早期诊断中的作用。方法 选取2013年10 月至2014 年12 月诊断为GBS 的32 例患儿进行神经电生理检查,同期选取30 例健康儿童为对照组,检测并分析两组正中神经和尺神经的腕部、肘部、Erb 点刺激复合肌肉动作电位(CMAP)及末端运动传导潜伏期(DML),正中神经F 波潜伏期,以及胫神经H 反射潜伏期。结果 病例组F 波和H 反射潜伏期均明显长于对照组(P<0.05)。32 例患儿中,24 例Erb 点波幅异常(75%),22 例Erb 点潜伏期异常(69%),20 例Erb 点传导阻滞(62%),病例组尺神经、正中神经 Erb 点CMAP 均小于对照组,而DML 均长于对照组(P<0.05);两组腕部和肘部的CMAP 及DML 差异无统计学意义(P>0.05)。结论 Erb 点刺激在GBS 早期诊断中有一定价值,可作为一项常规检查应用于GBS 的早期诊断。     

小儿运动神经传导正常值及发育规律

研究目的探讨小儿运动神经传导正常值及发育规律。研究方法１４９名０－１４岁儿童按年龄分为７组，成人组共１９人，年龄（２０－３５）岁。用神经诱发电位仪测量各受试者正中神经，尺神经，胫神经和腓神经的运动传导功能。结果６岁前神经动作电痊潜伏期随年龄增大而缩短，６岁后又渐延长，新生儿尺神经传导速度最快，其次为腓神经和正中神经，最慢为胫神经。出生后３个月，正中神经发育首先加快，３岁接近尺神经。胫神经于３个月－     

先天性马蹄内翻足的电生理学研究

目的　采用电生理检测手段 ,研究先天性马蹄内翻足患儿下肢神经和肌肉功能的变化 ,为有关该病病因学研究及其治疗方案的设计和疗效评价提供客观依据。方法　采用电生理检测手段 ,用丹麦产维迪KEYPOINT 4C诱发电位仪 ,对 42例临床确定为先天性马蹄内翻足的患儿进行了电生理检查。其中男 2 9例 ,女 1 3例 ,年龄 0 .5～ 1 2岁 ,平均 (2 6 .48± 36 .43)个月 ,身高 60～ 1 50cm ,平均 (80 .57± 2 2 .1 9)cm。其中双侧马蹄内翻足患儿 1 8例 ,单侧 2 4例。临床检查显示 ,足踝部呈马蹄内翻畸形 ,轻度 1 2例 1 4足 ,中度 1 7例 2 5足 ,重度 1 3例 2 1足。分别在窝、踝部阈值刺激腓总神经和胫神经 ,刺激频率 2Hz ,波宽 0 .2ms,分析时间 40ms,刺激强度 41～ 99mA ,平均 (85 .34± 2 1 .34)mA。在踝部伸趾短肌和展肌处记录肌肉的动作电位 ,并测量潜伏期和波幅 ,计算运动神经传导速度和潜伏速率。结果　 42例患儿 60侧患肢电生理检查发现 ,有 37例 53侧 (88.1 % )均有异常改变。其中有 8例 1 2侧 (1 9.1 % )患肢最大刺激下均未引出肌肉动作电位 ;8例 1 2侧 (1 9.1 % )患肢肢体腓总神经或胫神经运动神经传导速度、潜伏速率减慢 ;2 1例 2 9侧 (50 .0 % )患肢仅表现动作电位波幅下降。只有 5例 7侧 (1 1 .9% )电生     

短潜伏期体感诱发电位在慢性炎性脱髓鞘性多发性神经根神经病中的诊断作用

目的探讨短潜伏期体感诱发电位(SSEP)对慢性炎性脱髓鞘性多发性神经根神经病(CIDP)的诊断作用。方法 48例确诊或可能CIDP患儿及40例健康对照儿童进行肌电图神经传导和/或SSEP检查(健康对照仅行SSEP检查)。肌电图神经传导检查采用4道肌电图仪进行,包括至少4条运动神经和2条感觉神经;SSEP主要观察正中神经的N6(肘部电位),N13(颈髓电位),N20(皮质电位);胫神经的N8(腘窝电位),N22(腰髓电位),P39(皮质电位)。结果肌电图神经传导检测提示,48例患儿中35例运动、感觉神经均脱髓鞘,符合CIDP确诊标准;8例为感觉神经脱髓鞘,5例为轴突变性为主。40例患儿SSEP异常(未符合CIDP确诊标准的8例感觉受累患儿和5例继发轴突变性患儿的SSEP均异常),其中7例可见臂丛神经干和/或后根水平传导异常,33例同时有腰骶丛和/或后根损害。与健康对照相比,CIDP患儿N13、N22波幅潜伏期明显延长(P0.05)。结论 SSEP可用于CIDP辅助诊断,特别是对于感觉神经受累为主或继发轴突变性的CIDP患儿。     

脊髓栓系综合征的电生理检测特点及临床评价

目的评价脊髓栓系综合征（tethered cord syndrome，TCS）患儿的神经损伤。方法2001年5月42006年5月对105例TCS患儿行胫神经体感诱发电位（posterior tibial nerve somatosensory evoked potential study，PTN—SEP）和胫神经、腓总神经运动神经传导（motor conductive velocity，MCV）检测，以及盆底肌肌电图检查。结果TCS患儿皮层电位以及马尾电位潜伏期延长，腘窝处周围电位潜伏期无延长。胫神经MCV异常率34％，58％波幅降低，腓总神经MCV异常率24％，33％波幅降低。盆底肌肌电随着神经损伤程度的加重，由静息状态无自发电位，可见持续收缩电位，小力收缩运动单位电位波幅、时限、多相波升高，大力收缩募集相基本正常．发展到静息状态有自发电位，无或有极少量持续收缩动作电位，小力、大力收缩无动作电位。结论神经电生理检测对判断TCS患儿的神经损害程度及范围，了解预后具有重要的价值。     

肱骨髁上骨折伴移位时神经损伤的观察

复习肱骨髁上骨折中神经损伤的治疗经验以指导临床工作。共收治８６例肱骨髁上骨折伴神经损伤病例，其中桡神经损伤４７例次，尺神经损伤３例次，正中神经损伤５０例次。因Ｘ线片上均有骨折移位及明显的旋转和短缩，多数患儿采取了非手术治疗。随访的５０例神经损伤病例中，４６例（９２％）经非手术治疗，神经功能恢复。４例（８％）手术探查后恢复功能。因此，移位型肱骨髁上骨折伴神经损伤应以保守治疗为主。挠神经损伤的恢复可观察３个月，正中神经损伤可观察２个月，超过恢复时限后应积极进行肌电图检查和手术治疗。     

Moebius syndrome with total anomalous pulmonary venous connection

Moebius syndrome is characterized by congenital complete or partial facial nerve palsy with or without paralysis of cranial nerves and often associated with other malformations. Cardiac anomalies though known are very rare and till date only 4 cases have been reported. We present a case of Moebius syndrome with supracardiac total anomalous pulmonary venous connection which has not yet been reported in literature.     

神经营养素-3体外诱导新生鼠海马神经干细胞定向分化

目的探讨神经营养素-3（NT-3）对神经干细胞（NSCs）定向分化的影响。方法取24h龄新生鼠海马组织，以无血清培养基获得NSCs，随即分为实验和对照组。实验组用基础培养液加50g／L胎牛血清（FBS）和20μg／LNT-3诱导其分化；对照组用基础培养液加50g／LFBS。用免疫荧光及流式细胞仪法检测分化得到神经元特异性烯醇化酶（NSE）神经元及阳性神经元比例。结果随着分化培养时间延长，二组NSE阳性神经元渐增加；实验组NSE阳性神经元的比例在分化培养第3天达到高峰，约63％，对照组仅29％，二组比较差异有统计学意义（P〈0．05）。结论NT-3体外促进NSCs向神经元定向分化，并可提高其分化比例。     

The effect of the genitofemoral nerve division on the contralateral descended testis in the unilateral cryptorchidism model

It is widely accepted that there are degenerative changes and decreased spermatogenesis in the contralateral descended testis (CDT) in unilateral undescended testis (UUDT). While some investigators have postulated that the mechanism may be related to primary (congenital) or secondary (autoimmune, vascular, and neural) events, the exact mechanism of the damage to the CDT is still unknown. The present study was planned to investigate the role of the genitofemoral nerve (GFN) on the changes in the CDT. Forty male Wistar albino rats were divided into four groups of 10 each. During the newborn period a UUDT model was created and at the age of 30 days ipsilateral GFN division was done (group A). In addition, UUDT with intact GFN (group B), divided right GFN with bilateral scrotal testes (group C), and control (group D) groups were formed. When the animals reached early adulthood, they were killed and the testes were removed. Mean seminiferous-tubular diameter (STD) and germinal-ephitelium maturity was determined using modified Johnson testicular biopsy scores (TBS). The mean STD and TBS of the study groups did not show any differences suggesting that ipsilateral division of the GFN has no effect on the CDT in the UUDT model. Accepted: 11 August 2000     

Division of the genitofemoral nerve in normal scrotal testicular rats

The role of the genitofemoral nerve (GFN) on testicular descent has been clearly shown. It has also been suggested that in unilateral cryptorchid rats, after division of the ipsilateral GFN fertility rates are higher, i.e., transection of the GFN prevents contralateral testicular damage, but the mechanism is unclear. The purpose of this study was to investigate the effect of dividing the GFN on the normal scrotal testes. Thirty male Wistar albino rats were divided into three groups: group A, transection of right GFN; group B, bilateral transection of the GFN; and group C, sham operations, all at the age of 30 days. The animals were killed at 90 days of age and the testes were removed. Each excised testis was weighed and fixed for histological studies. Mean seminiferous tubular diameter was measured and germinal epithelium maturity was determined using the modified Johnsen testicular-biopsy score. In all groups, all three parameters were similar, suggesting that division of the GFN had no effect on normal testes. Accepted: 12 January 2000     

儿童正中神经损伤81例报告

目的：对８１例８４条儿童正中神经损伤进行回顾性研究，讨论其诊断和处理问题。方法：伤于腋部３条，上臂１０条，肘部３０条，前臂１４条，腕部２４条，掌部３条；完全离断５３条，部分断裂４条，挫伤或压迫变性２７条；开放伤５６条，闭合伤２８条。采用神经外膜对端吻合术５１条，自体神经移植术４条，神经外松解术１５条，因毁损未处理３条，骨折复位外固定观察１１条。结果：随访５０条，１～２８（平均７．８）年，优良率为８８％。结论：本组有１４例漏诊，为避免漏诊应详细问病史、反复检查。神经开放伤（火器伤等除外）应一期手术探查修复神经，骨折合并伤观察１～３个月，晚期神经伤也要力争手术修复神经。神经断裂以外膜缝合为主，神经受压或变性以神经外松解术为主，拇指对掌功能恢复不佳时用小指外展肌重建拇指对掌功能。     

Thickening and enhancement of multiple cranial nerves in conjunction with cystic white matter lesions in early infantile Krabbe disease

We present serial MR findings in a child ultimately diagnosed with the early infantile form of Krabbe disease. MR showed typical features of Krabbe disease including cerebellar and brainstem hyperintensity, periventricular and deep white matter hyperintensity, and cerebral atrophy. In addition, the combination of both enlargement and enhancement of multiple cranial nerves in conjunction with unusual cystic lesions adjacent to the frontal horns of the lateral ventricles was previously unreported and expands the spectrum of imaging findings in early Krabbe disease.     

Fluorescent anterograde labelling of the genitofemoral nerve shows that it supplies the scrotal region before migration of the gubernaculum

The genitofemoral nerve (GFN) contains a sexually dimorphic neuropeptide transmitter, calcitonin generelated peptide (CGRP). It has been proposed that release of CGRP from the nerve may mediate testicular descent. The aim of this study was to determine the course of the GFN in order to see if CGRP-containing fibres reached the future scrotum before gubernacular migration occurs, since this arrangement would be expected if the nerve controls gubernacular migration by CGRP release. Fluorescent anterograde labelling of the cut GFN in young rats using diamidinophenyl indole (DAPI) or Fast Blue was performed to determine the distal course of the nerve. On frozen serial sections, the nerve was found running posterolateral to the developing spermatic cord in the inguinal canal, then distally on the surface of the cremaster muscle. It then turned cranially to enter the gubernaculum from its distal attachment while some branches continued past the gubernaculum to end in the skin of the future scrotum. Immunoperoxidase staining for CGRP showed labelling in all GFN fibre bundles, including those reaching the scrotum. The course of the nerve with its sexually dimorphic neurotransmitter, CGRP, suggests that the nerve may influence the direction of gubernacular migration from the groin into the scrotum. Offprint requests to: J. M. Hutson     

Optic nerve head infiltration in acute leukemia in children: An indication for emergency optic nerve radiation therapy

Two pediatric patients with acute leukemia who developed optic nerve head leukemic infiltration are presented. In one patient both eyes were involved at diagnosis as well as her central nervous system. Despite systemic and intrathecal chemotherapy she lost her vision within a few weeks. Cranial irradiation at that point could not reverse this outcome. In the second patient optic nerve head infiltration was found a few months after diagnosis, treated promptly with cranial irradiation and her vision was saved. Her central nervous system (CNS) was not involved at any time. It is stressed that ocular complaints including eye pain or blurred vision in the pediatric patient with leukemia should be investigated without delay by an ophthalmologist. In the young child these complaints may be absent and change in the visual behavior should then alert the pediatric oncologist for possible ocular problems. If optic nerve head leukemic infiltration is diagnosed and promptly treated with emergency radiation, vision can be salvaged. © 1996 Wiley-Liss, Inc.     

交感神经皮肤反应在糖尿病患儿周围神经病早期诊断中的应用

目的探讨交感神经皮肤反应(SSR)在儿童糖尿病周围神经病交感神经纤维病变早期诊断的应用价值。方法对35例1型糖尿病(T1DM)儿童和30例健康儿童四肢分别进行电刺激SSR检测,测量每个肢体的平均起始潜伏期和峰-峰波幅,并比较2组各肢体的SSR起始潜伏期及峰-峰波幅差异。同时对T1DM组儿童进行神经传导速度(NCV)的检测,并对NCV检测异常率与SSR检测异常率进行比较。另外,还对有酮症酸中毒(DKA)史和无DKA史的2组患儿SSR检测异常结果进行比较。结果 T1DM组患儿各肢体SSR起始潜伏期均较健康对照组明显延长(Pa<0.05)。健康对照组30例四肢均可引出SSR波形,T1DM组31例患儿四肢均可引出SSR波形;4例患儿至少有一肢不能引出SSR波形(11.4%),其中2例患儿四肢均未引出SSR波形(5.7%);21例(60%)患儿至少有一肢SSR缺失或起始潜伏期异常,其中15例(71.4%)有DKA史,高于无DKA患儿异常检出率(6例,42.9%),但2组比较无统计学差异。T1DM组患儿NCV检测异常10例(28.6%),较SSR起始潜伏期的检出异常率低,两者比较差异无统计学意义(P>0.05)。结论 SSR检测可早期发现糖尿病周围神经病交感神经小纤维病变,较NCV敏感。