儿童吉兰-巴雷综合征诊治分析

急性感染性多发神经根神经炎又称吉兰-巴雷综合征(GBS),是一种由病毒感染等前驱疾病所诱发的自身免疫性疾病,其基本的病理特征为炎性脱髓鞘.临床特点是急性起病,渐进性、对称性、弛缓性肢体麻痹,病程早期可有不同程度的感觉障碍,严重者常伴有颅神经麻痹及呼吸肌麻痹[1].     

儿童吉兰-巴雷综合征临床诊治分析

急性感染性多发神经根神经炎（acutc infcctious polyradiculoncuritis,GBS）又称吉兰-巴雷综合征,是一种由病毒感染等前驱疾病所诱发的自身免疫性疾病,其基本病理特征为炎性脱髓鞘。临床特点是急性起病,渐进性、对称性、弛缓性肢体麻痹,病程早期可有不同程度的感觉障碍,严重者常伴有脑神经麻痹及呼吸肌麻痹。     

儿童吉兰——巴雷综合征临床分析

急性感染性多发神经根神经炎(acutc infcctiouspolyradiculoncuritis)又称吉兰-巴雷综合征(简称GBS),是一种由病毒感染等前驱疾病所诱发的自身免疫性疾病,其基本的病理特征为炎性脱髓鞘。临床特点是急性起病,渐进性、对称性、弛缓性肢体麻痹,病程早期可有不同程度的感觉障碍,严     

感觉共济失调型吉兰-巴雷综合征二例

1 临床资料 病例1:男,59岁,因"双下肢麻木1个月,双上肢麻木2周,伴有步态不稳、味觉减退、闭目困难、流泪、面部表情僵硬、言语含糊"于2007-12-25入院.     

吉兰-巴雷综合征患者护理体会

急性炎症性脱髓鞘性突发性神经病又称吉兰-巴雷综合征(GBS),是可能与感染有关和免疫机制参与的急性(或亚急性)特发性多发性神经病[1],以青壮年多见.6年来我科共收治患者36例, 现将护理体会总结如下.     

大剂量免疫球蛋白治疗吉兰-巴雷综合征2l例临床观察

目的观察大剂量静滴免疫球蛋白(IVIg)治疗吉兰-巴雷综合征(GBS)的疗效。方法2l例确诊病人于首次发病后10天内接受IVIg治疗．剂量为0．4g·kg-1．D-1,连续5天为一疗程,结果神经功能缺损评分治疗前后差异具有高度显著性(P<0．01)。结论IVIg治疗GBS具有安全、高效、副作用少等优点,值得进一步推广。     

吉兰-巴雷综合征合并中枢神经系统损害一例

1临床资料患者女,44岁,因四肢麻木、无力26 d于2004-09-17入院。26 d前受凉后出现双下肢麻木、无力,8d前出现双手麻木、无力,偶有呛咳,曾在外院按吉兰-巴雷综合征(GBS)接受维生素等药物治疗,但病情继续加重。入院神经科查体:意识清楚,声音低哑,言语欠流利;可见明显水平眼震,双瞳孔等大等圆,光反应灵敏;双额及面纹浅,双眼闭合力弱;双软腭上抬力弱,咽反射迟钝,伸舌居中;四肢肌力Ⅱ~Ⅳ级,肌张力低,腱反射弱,病理征阴性,无深浅感觉减退。5次脑脊液检查结果显示,脑脊液压力除第1次正常外均增高(200~270 mmH2O),白细胞数除第1次异常(20×106/L)…     

18例吉兰-巴雷综合征误诊分析

吉兰-巴雷综合征(GBS)是一类周围神经系统脱髓鞘病变,具体病因不明,临床症状以进行发展的对称性四肢软瘫为主要表现,其中一部分病人伴有脑神经损害、异常感觉和植物神经功能障碍。对于典型的吉兰-巴雷综合征病例的诊     

吉兰-巴雷综合征的MRI诊断

目的 分析吉兰-巴雷综合征(OBS)患者的MRI扫描特征和限度. 方法对15例GBS患者(急性14例、慢性1例)行MRI平扫和增强后脂肪抑制TIWI扫描,观察椎管内周围神经的MRI表现及其与临床特征的关系.结果 MRI平扫显示8例急性患者马尾神经不同程度的增粗,T1WI为中等信号,T2W1为等或略高信号,1例慢性患者全脊椎的脊神经和马尾神经增粗.增强扫描显示14例急性患者T<,8>以下出现部分脊神经和马尾神经不同程度的增粗、强化,其中2例伴有部分颈脊神经增粗、强化,2例伴有部分颅神经增粗、强化.1例慢性患者全脊椎的脊神经、马尾神经和部分颅神经增粗、强化;全部患者出现双下肢乏力,其中双下肢瘫痪9例,MRI增强扫描均显示马尾神经明显强化(100%);双上肢乏力7例,MRI阳性3例(43%);颅神经功能障碍6例,MRI阳性3例(50%).结论 MRI可以敏感检测GBS患者的马尾神经病变,但颈神经和颅神经受累易漏诊.     

吉兰-巴雷综合征机械通气临床分析

回顾分析作者科室1 7年3 1 2例吉兰-巴雷综合征(Guillain-Barre syndrome,GBS)患者中5 6例呼吸衰竭需要机械通气患者的临床特点,并探讨机械通气的影响因素。1　资料和方法　5 6例GBS呼吸肌麻痹需呼吸机辅助呼吸患者,男3 5例、女2 1例,中位年龄3 4.5岁(2～66岁)。调查患者机械通气前的病程、机械通气时间、机械通气时的并发症、预后和死亡原因。进行Cox回归分析。2　结果2 .1　机械通气的时间和并发症:从出现首发症状至需机械通气的中位时间为7d(1～96d)。47例气管切开,9例经口或经鼻插管,呼吸机辅助呼吸中位时间为2 7d(1～1 5 4d) ,部分…     

淋巴血浆置换治疗Guillain-Barre综合征的疗效观察

目的 观察淋巴血浆置换(LPE)治疗Guillain-Barre综合征(GBS)的疗效.方法 对34例GBS患者(LPE组)在常规治疗基础上加用LPE治疗,观察肌力开始恢复时间、治疗前后肌力评分差值及临床疗效和不良反应,并与17例加用血浆置换(TPE)治疗的GBS患者(TPE组)进行比较.结果 LPE组共接受LPE 51次,平均1.5次/例;TPE组共接受TPE治疗33次,平均1.9次/例.LPE组治疗后肌力开始恢复时间为(12.74±7.18)d,显著早于TPE组[(24.35±14.22)d](P<0.05);治疗前后的肢体肌力评分差值大于TPE组(均P<0.05),疗效为进步者(24例)多于TPE组(7例),无效者(2例)少于TPE组(8例),总有效率(94.12%)高于TPE组(52.94%)(P<0.001);平均住院(23.47±12.91)d,短于TPE组的(48.12±39.08)d;差异有统计学意义(P<0.05).LPE组不良反应有荨麻疹(4例)和低血压(2例),均轻微,对症处理后缓解,不良反应发生率为11.8%,与TPE组(21.2%)比较差异无统计学意义(P>0.05). 结论 LPE治疗GBS疗效显著,不良反应少,是一种安全、有效的治疗方法,值得临床推广应用.     

Character of diaphragm compound muscle action potential and phrenic nerve conduction time in patients with obstructive sleep apnea-hypopnea syndrome

BACKGROUND: Both hypoxia and carbon dioxide retention can damage phrenic nerve and muscle conduction, as well as diaphragm function. Diaphragm compound muscle action potential and phrenic nerve conduction time are reliable indicators for measuring phrenic nerve and diaphragm function.OBJECTIVES: To verify the hypothesis that changes of phrenic nerve conduction time (PNCT) and diaphragm compound muscle action potential (CMAP) in obstructive sleep apnea-hypopnea syndrome (OSAHS) patients might contribute to the decline of phrenic nerve and diaphragm function. PNCT and CMAP were measured with multipair esophageal electrodes combined with unilateral magnetic stimulation.DESIGN, TIME AND SETTING: Case controlled study. The experiment was carried out in Guangzhou Institute of Respiratory Disease, Guangzhou Medical College, from June 2005 to April 2006.PARTICIPANTS: Twenty seven OSAHS patients and eight primary snoring subjects from Guangzhou Institute of Respiratory Disease, Guangzhou Medical College were recruited and all subjects were diagnosed by polysomnography (PSG). Sixteen healthy, non-snoring subjects in the hospital for medical examination during the same time period were selected as the control group.METHODS: Esophageal electrodes, made by Guangzhou Institute of Respiratory Disease, combined with unilateral magnetic stimulation, were used to measure PNCT and CMAP of all subjects. PNCT was defined as the time from stimulation artifact to the onset of CMAP and diaphragm CMAP amplitude was measured from peak to peak. Oxygen desaturation index and apnea-hypopnea index were measured using PSG, and their relevance to PNCT and CMAP were analyzed. PNCT and CMAP in five OSAHS patients were repeatedly measured after effective nasal continuous positive airway pressure treatment for more than 2 months.MAIN OUTCOME MEAAURES: (1) PNCT and diaphragm CMAP of suhjects in each group. (2) Relevance of oxygen desaturation index and apnea-hypopnea index to PNCT and CMAP. (3) Changes of PNCT and CMAP of OSAHS patients before and after treatment.RESULTS: All subjects were included in the analyzed results. (1) PNCT of the OSAHS group was significantly longer compared to that of the control and primary snore groups, while CMAP of the OSAHS group was significantly lower (P<0.05). (2) PNCT and CMAP recorded from both sides correlated significantly with oxygen desaturation index and with apnea-hypopnea index (P<0.01). (3) PNCT shortened signiticantly after effective nasal continuous positive airway pressure treatment for more than 2 months (P<0.05).CONCLUSION: Prolongation of PNCT and decrease of CMAP might contribute to the decline of phrenic nerve and diaphragm function caused by repeated nocturnal hypoxia and carbon dioxide retention. The impairment of the phrenic nerve might also decrease diaphragm function.     

Character of diaphragm compound muscle action potential and phrenic nerve conduction time in patients with obstructive sleep apnea-hypopnea syndrome☆◇

BACKGROUND： Both hypoxia and.carbon dioxide retention can damage phrenic nerve and muscle conduction, as well as diaphragm function. Diaphragm compound muscle action potential and phrenic nerve conduction time are reliable indicators for measuring phrenic nerve and diaphragm function. OBJECTIVES： To verify the hypothesis that changes of phrenic nerve conduction time （PNCT） and diaphragm compound muscle action potential （CMAP） in obstructive sleep apnea-hypopnea syndrome （OSAHS） patients might contribute to the decline of phrenic nerve and diaphragm function. PNCT and CMAP were measured with multipair esophageal electrodes combined with unilateral magnetic stimulation. DESIGN, TIME AND SETTING： Case controlled study. The experiment was carried out in Guangzhou Institute of Respiratory Disease, Guangzhou MediCal College, from June 2005 to April 2006. PARTICIPANTS： Twenty seven OSAHS patients and eight primary snoring subjects from Guangzhou Institute of Respiratory Disease, Guangzhou Medical College were recruited and all subjects were diagnosed by polysomnography （PSG）. Sixteen healthy, non-snoring subjects in the hospital for medical examination during the same time period were selected as the control group. METHODS： Esophageal electrodes, made by Guangzhou Institute of Respiratory Disease, combined with unilateral magnetic stimulation, were used to measure PNCT and CMAP of all subjects. PNCT was defined as the time from stimulation artifact to the onset of CMAP and diaphragm CMAP amplitude was measured from peak to peak. Oxygen desaturation index and apnea-hypopnea index were measured using PSG, and their relevance to PNCT and CMAP were analyzed. PNCT and CMAP in five OSAHS patients were repeatedly measured after effective nasal continuous positive airway pressure treatment for more than 2 months. MAIN OUTCOME MEASURES： （1） PNCT and diaphragm CMAP of subjects in each group. （2） Relevance of oxygen desaturation index and apnea-hypopnea index to PNCT and CMAP. （3） Changes of     

吉兰-巴雷综合征患者脑脊液中S-100b、MBP的检测及意义

目的 研究吉兰-巴雷综合征(green-barre syndrome,GBS)患者脑脊液中S-100 b和髓鞘碱性蛋白(MBP)的含量变化,探讨S-100 b及MBP在GBS发病过程中的作用以及与病情严重程度的关系.方法 采用酶联免疫吸附法(ELISA)测定32例不同临床分级的GBS患者(GBS组),30例其他神经系统...     

大剂量免疫球蛋白治疗Guinain-Barre综合征的疗效观察

目的 观察大剂量静脉注射免疫球蛋白(IVIG)治疗Guillain-Barre综合征(GBS)的疗效.方法 将34例急性GBS患者随机分为WIG治疗组(IVIG组)和常规治疗组(对照组)(各17例),IVIG组用人免疫球蛋白0.4 g/(kg·d)静脉注射,连续5 d;其他治疗两组相同.治疗21 d后评定临床疗效.结果 IVIG组治愈率(52.9%)及总有效率(100%)明显高于对照组(11.8%,76.5%)(均P＜0.05),且四肢肌力、呼吸肌瘫痪、感觉障碍、脑神经损害开始改善的时间明显短于对照组(均P＜0.001).结论 大剂量IVIG治疗GBS有显著疗效,无明显不良反应.     

Albumin levels as a biomarker for second Intravenous Immunoglobulin (IVIG) treatment in Guillain-Barre syndrome (GBS)

BackgroundPatients with GBS may develop hypoalbuminemia following treatment with Intravenous Immunoglobulin (IVIG), which is related to a poorer outcome. This report presents a patient with GBS and his clinical response to two courses of IVIG treatments in association with his albumin level.Case report: A previously healthy 21-year-old male was admitted to the GICU due to GBS with severity grade 5 (required assisted ventilation). IVIG treatment was initiated. Over the next two weeks there was no clinical improvement and Albumin level dropped from 4.5 gr/dL to a nadir of 2.3 gr/dL. A second course of IVIG was initiated. After initiation of the second course the patient’s albumin began rising to 3.0 gr/dL and a clinical improvement followed this rise. Subsequently, he was weaned from mechanical ventilation within a few days.ConclusionsWhen considering a second course of IVIG treatment, serum albumin levels may be considered a biomarker as part of the decision algorithm.     

Influence of reference electrode position on the compound muscle action potential

ObjectiveWhen the compound muscle action potential (CMAP) is recorded in motor nerve conduction studies, the reference (E2) electrode can make a significant contribution to the CMAP. This study investigates the E2 recorded signal and its effect on CMAP measurements when E2 electrode is placed at different sites.MethodsThe CMAP was recorded using the active electrode on the muscle belly and 4 different E2 electrodes placed at distal and proximal sites. The signal recorded by each electrode was also measured using a reference electrode on the contralateral limb. Signals were recorded from the abductor pollicis brevis, abductor digiti minimi, tibialis anterior and biceps muscles.ResultsThe E2 recorded a smaller signal when it was placed near or off the proximal tendon or muscle origin. This affected CMAP latency, duration for tibialis anterior. Contrary to expectation, initial upward deflection was noted for E2 signal.ConclusionA proximal E2 position records a lower volume conducted signal and yields a CMAP that is more representative of the muscle over which the E1 (active) electrode is placed.SignificanceThe proposed ‘Proximal E2’ montage may be better suited to assess pathology.     

Correlation between compound muscle action potential amplitude and duration in axonal and demyelinating polyneuropathy

Objective

To get a better understanding of pathophysiology in polyneuropathies (PNPs) by correlating compound muscle action potential (CMAP) amplitude with duration.

Methods

A total of 145 motor nerve conduction studies (MNCS) in 53 axonal and 132 MNCS in 45 demyelinating PNPs were analyzed. Peroneal and tibial MNCS were done by surface stimulation while for median and ulnar nerves near nerve or surface stimulations were used. CMAP amplitude and duration were compared in axonal and demyelination PNPs. Relationships between amplitude and duration of distally and proximally evoked CMAP were examined using regression analysis.

Results

CMAP amplitude was lower and duration was increased in all examined nerves in demyelinating PNPs than in axonal PNPs. In demyelinating PNPs, an inverse linear correlation between amplitude and duration was seen in distally and proximally evoked CMAP in all examined nerves. In axonal PNPs, there was no correlation in any of the nerves neither in distally nor in proximally evoked CMAP.

Conclusions

Distal CMAP duration and the relationship between CMAP amplitude and duration show supplementary electrodiagnostic potential in demyelinating PNPs.

Significance

More knowledge about the relation between amplitude and duration in axonal lesions and demyelination may help to reveal the pathophysiology in PNPs. Significant correlation between amplitude and duration in demyelination may suggest that the severe decrease in amplitude in demyelinating PNPs is probably due to the increase in duration secondary to temporal dispersion.     

Impact of stimulus duration on motor unit thresholds and alternation in compound muscle action potential scans

ObjectiveTo investigate the impact of stimulus duration on motor unit (MU) thresholds and alternation within compound muscle action potential (CMAP) scans.MethodsThe stimulus duration (0.1, 0.2, 0.6, and 1.0 ms) in thenar CMAP scans and individual MUs of 14 healthy subjects was systematically varied. We quantified variability of individual MU’s thresholds by relative spread (RS), MU thresholds by stimulus currents required to elicit target CMAPs of 5% (S5), 50% (S50) and 95% (S95) of the maximum CMAP, and relative range (RR) by 100*[S95-S5]/S50. We further assessed the strength-duration time constant (SDTC). Experimental observations were subsequently simulated to quantify alternation.ResultsRS, unaffected by stimulus duration, was 1.65% averaged over all recordings. RR increased for longer stimulus duration (11.4% per ms, p < 0.001). SDTC shortened with higher target CMAPs (0.007 ms per 10% CMAP, p < 0.001). Experiments and simulations supported that this may underlie the increased RR. A short compared to long stimulus duration recruited relative more MUs at S50 (more alternation) than at the tails (less alternation).ConclusionsThe stimulus duration significantly affects MU threshold distribution and alternation within CMAP scans.SignificanceStimulation settings can be further optimized and their standardization is preferred when using CMAP scans for monitoring neuromuscular diseases.