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Presence of antibodies against low-density lipoprotein receptor-related protein 4 and impairment of neuromuscular junction in a Chinese cohort of amyotrophic lateral sclerosis
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作者 Lin Lei Xin-Ming Shen +8 位作者 Shu-Yan Wang Yan Lu Suo-Bin Wang Hai Chen Zheng Liu Ya-Sheng Ouyang Jian-Ying Duo Yu-Wei Da Zhi-Guo Chen 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第12期1487-1489,共3页
Amyotrophic lateral sclerosis (ALS) is a heterogeneous disorder characterized by a loss of upper and lower motor neurons with neither clear pathogenesis nor effective treatment. Thus, potential biomarkers are needed t... Amyotrophic lateral sclerosis (ALS) is a heterogeneous disorder characterized by a loss of upper and lower motor neurons with neither clear pathogenesis nor effective treatment. Thus, potential biomarkers are needed to classify the disease and find new drug targets. Previous studies have shown that auto-antibodies against the low-density lipoprotein receptor-related protein 4 (LRP4), called the anti-LRP4 antibodies, are found in ~23% patients of Greek and Italian ALS cohorts,[1] and in 10% of the American ALS population.[2] Anti-LRP4 antibodies were previously identified in myasthenia gravis (MG), the most common neuromuscular junction (NMJ) disorder, and were shown to cause NMJ abnormality in animal studies.[3] Here, we studied anti-LRP4 antibodies in Chinese patients and investigated the correlation between anti-LRP4 antibodies and abnormal neuromuscular transmission in ALS. 展开更多
关键词 ANTIBODIES AGAINST amyotrophic lateral SCLEROSIS NEUROMUSCULAR junction
Study on variation trend of repetitive nerve stimulation waveform in amyotrophic lateral sclerosis
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作者 Li-Lan Fu He-Xiang Yin +1 位作者 Ming-Sheng Liu Li-Ying Cui 《中华医学杂志:英文版》 SCIE CAS CSCD 2019年第5期542-550,共9页
Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower neurons with no effective cure. Electrophysiological studies have found decremental responses d... Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease involving both upper and lower neurons with no effective cure. Electrophysiological studies have found decremental responses during low-frequency repetitive nerve stimulation (RNS) except for diffused neurogenic activities. However, the difference between ALS and generalized myasthenia gravis (GMG) in terms of waveform features is unclear. In the current study, we explored the variation trend of the amplitudes curve between ALS and GMG with low-frequency, positive RNS, and the possible mechanism is discussed preliminarily. Methods: A total of 85 ALS patients and 41 GMG patients were recruited. All patients were from Peking Union Medical College Hospital (PUMCH) between July 1,2012 and February 28,2015. RNS study included ulnar nerve, accessory nerve and facial nerve at 3 Hz and 5 Hz stimulation. The percentage reduction in the amplitude of the fourth or fifth wave from the first wave was calculated and compared with the normal values of our hospital. A 15% decrease in amplitude is defined as a decrease in amplitude. Results: The decremental response at low-frequency RNS showed the abnormal rate of RNS decline was 54.1%(46/85) in the ALS group, and the results of different nerves were 54.1 %(46/85) of the accessory nerve, 8.2%(7/85) of the ulnar nerve and 0%(0/85) of the facial nerve stimulation, respectively. In the GMG group, the abnormal rate of RNS decline was 100%(41/41) at low-frequency RNS of accessory nerves. However, there was a significant difference between the 2 groups in the amplitude after the sixth wave. Conclusions: Both groups of patients are able to show a decreasing amplitude of low-frequency stimulation RNS, but the recovery trend after the sixth wave has significant variation. It implies the different pathogenesis of NMJ dysfunction of these 2 diseases. 展开更多
关键词 Amyotrophic lateral SCLEROSIS Generalized MYASTHENIA GRAVIS NEUROMUSCULAR junction REPETITIVE nerve stimulation
Muscle Fiber Type Changes in Lumbrical Muscles at Early Stages of Chronic Nerve Compression 预览
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作者 Zhen-yu LIU Jiang-hai CHEN Zhen-bing CHEN 《当代医学科学(英文)》 SCIE CAS 2019年第1期59-66,共8页
Chronic nerve compression(CNC)neuropathy is a common disease in the clinic and provokes paraesthesia,or numbness at early stage.The changes in muscle fiber composition and motor nerve terminal morphology in distal mus... Chronic nerve compression(CNC)neuropathy is a common disease in the clinic and provokes paraesthesia,or numbness at early stage.The changes in muscle fiber composition and motor nerve terminal morphology in distal muscles were studied in this study.A well-established CNC model was used to assess the changes in the muscles.Behaviors were measured by von Frey filament test The myosin heavy chain isoforms and neuromuscular junctions(NMJs)were stained by immunofluorescence to show the muscle fiber types composition and motor nerve terminals morphologic changes in the flexor digitorum longus(FDL)and lumbrical muscle.The fiber cross-sectional areas of different muscle fiber types were measured.The small-fiber degeneration of cutaneous nerve fibers was examined by detecting the protein gene product 9.5(PGP9.5)with immunofluorescence.At 2nd month after compression,the proportion of type I and type IIB fibers was markedly decreased,and that of type n A fibers was increased in the lumbrical muscle.There was no significant change in composition of muscle fiber types in FDL and NMJ morphology of FDL and lumbrical muscles.Intra-epidermal nerve fibre density(IENFD)declined at 2nd month after the compression.Our study reveals the morphological changes of the FDL and lumbrical muscle at an early stage of CNC.These findings may be helpful to understand muscle damage and pathophysiological development of the nerve compression,and provide new evidence for early treatment of CNC. 展开更多
关键词 CHRONIC NERVE compression NEUROPATHY CARPAL tunnel syndrome lumbrical muscles intra-epidermal NERVE fiber density NEUROMUSCULAR junctions
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Applying the Socio-Ecological Model to barriers to implementation of ACL injury prevention programs: A systematic review
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作者 Rima L.Bogardus Ryan J.Martin +1 位作者 Alice R.Richman Anthony S.Kulas 《运动与健康科学:英文版》 SCIE 2019年第1期8-16,共9页
Background: Preventing anterior cruciate ligament(ACL) injuries is important to avoid long-term adverse health consequences. Identifying barriers to implementation of these prevention programs is crucial to reducing t... Background: Preventing anterior cruciate ligament(ACL) injuries is important to avoid long-term adverse health consequences. Identifying barriers to implementation of these prevention programs is crucial to reducing the incidence of these injuries. Our purpose was to identify barriers of implementation for ACL injury prevention programs and suggest mechanisms for reducing the barriers through application of a SocioEcological Model(SEM).Methods: Studies investigating ACL prevention program effectiveness were searched in Medline via PubMed and the Cochrane Library, and a subsequent review of the references of the identified articles, yielded 15 articles total. Inclusion criteria encompassed prospective controlled trials, published in English, with ACL injuries as the primary outcome. Studies were independently appraised by 2 reviewers for methodological quality using the PEDro scale. Barriers to implementation were identified when reported in at least 2 separate studies. A SEM was used to suggest ways to reduce the identified barriers.Results: Five barriers were identified: motivation, time requirements, skill requirements for program facilitators, compliance, and cost. The SEM suggested ways to minimize the barriers at all levels of the model from the individual through policy levels.Conclusion: Identification of barriers to program implementation and suggesting how to reduce them through the SEM is a critical first step toward enabling ACL prevention programs to be more effective and ultimately reducing the incidence of these injuries. 展开更多
关键词 Anterior CRUCIATE LIGAMENT INJURY PREVENTION Barriers to IMPLEMENTATION NEUROMUSCULAR training programs Socio-Ecological Model Sports INJURY PREVENTION
Neuromuscular fatigue and recovery profiles in individuals with intellectual disability
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作者 Rihab Borji Firas Zghal +3 位作者 Nidhal Zarrouk Vincent Martin Sonia Sahli Haithem Rebai 《运动与健康科学:英文版》 SCIE 2019年第3期242-248,共7页
Purpose:This study aimed to explore neuromuscular fatigue and recovery profiles im individuals with intellectual disability(ID)after exhausting submaximal contraction.Methods:Ten men with ID were compared to 10 men wi... Purpose:This study aimed to explore neuromuscular fatigue and recovery profiles im individuals with intellectual disability(ID)after exhausting submaximal contraction.Methods:Ten men with ID were compared to 10 men without ID.The evaluation of neuromuscular function consisted in brief(3 s)isometric maximal voluntary contraction(IMVC)of the knee extension superimposed with electrical nerve stimulation before,immediately after,and during33 min after an exhausting submaximal isometric task at 15%of the IMVC.Force,voluntary activation level(VAL),potentiated twitch(Ptw),and electromyography(EMG)signals were measured during IMVC and then analyzed.Results:Individuals with ID developed lower baseline IMVC,VAL,Ptw;and RMS/Mmax ratio(root-mean-square value normalized to the maximal peak-to-peak amplitude of the M-wave)than controls(p<0.05).Nevertheless,the time to task failure was significantly longer in ID vs.controls(p<0.05).The 2 groups presented similar IMVC decline and recovery kinetics after the fatiguing exercise.However.individuals with ID presented higher VAL and RMS/Mmax ratio declines but lower Ptw decline compared to those without ID.Moreover,individuals with ID demonstrated a persistent central fatigue but faster recovery from peripheral fatigue.Conclusion:These differences in neuromuscular fatigue profiles and recovery kinetics should be acknowledged when prescribing training programs for individuals with ID. 展开更多
关键词 Central FATIGUE INTELLECTUAL DISABILITY NEUROMUSCULAR FATIGUE Peripheral FATIGUE SUSTAINED sub-maximal exercise TWITCH interpolation technique
Amyotrophic lateral sclerosis as a synaptopathy 预览
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作者 Matthew J. Fogarty 《中国神经再生研究:英文版》 SCIE CAS CSCD 2019年第2期189-192,共4页
The synapse is an incredibly specialized structure that allows for the coordinated communication of information from one neuron to another. When assembled into circuits, steady streams of excitatory and inhibitory syn... The synapse is an incredibly specialized structure that allows for the coordinated communication of information from one neuron to another. When assembled into circuits, steady streams of excitatory and inhibitory synaptic activity shape neural outputs. At the organismal level, ensembles of neural networks underlie behavior, emotion and memory. Disorder or dysfunctions of synapses, a synaptopathy, may underlie a host of developmental and degenerative neurological conditions. There is a possibility that amyotrophic lateral sclerosis may be a result of a synaptopathy within the neuromotor system. To this end, particular attention has been trained on the excitatory glutamatergic synapses and their morphological proxy, the dendritic spine. The extensive detailing of these dysfunctions in vulnerable neuronal populations, including corticospinal neurons and motor neurons, has recently been the subject of original research in rodents and humans. If amyotrophic lateral sclerosis is indeed a synaptopathy, it is entirely consistent with other proposed pathogenic mechanisms - including glutamate excitotoxicity, accumulation of misfolded proteins and mitochondrial dysfunction at distal axon terminals (cortico-motor neuron and neuromuscular). Further, although the exact mechanism of disease spread from region to region is unknown, the synaptopathy hypothesis is consistent with emerging die-forward evidence and the prion-like propagation of misfolded protein aggregates to distant neuronal populations. Here in this mini-review, we focus on the timeline of synaptic observations in both cortical and spinal neurons from different rodent models, and provide a conceptual framework for assessing the synaptopathy hypothesis in amyotrophic lateral sclerosis. 展开更多
关键词 MOTOR neuron MOTOR cortex CORTICOSPINAL EXCITOTOXICITY SYNAPTIC transmission DENDRITES dendriticspines NEUROMUSCULAR junction
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超声引导下腹横肌平面阻滞对妇科腹腔镜手术麻醉效果及术后早期镇痛的影响
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作者 沈莉莉 朱俊峰 《中国基层医药》 CAS 2019年第2期183-185,共3页
目的观察超声引导下腹横肌平面(TAP)阻滞对妇科腹腔镜手术麻醉效果及术后镇痛的影响。方法选择2015年1月至2017年7月在上海市嘉定区南翔医院择期全身麻醉下行妇科腹腔镜手术患者60例,年龄46~68岁,美国麻醉医师协会(ASA)分级Ⅰ~Ⅱ级。采... 目的观察超声引导下腹横肌平面(TAP)阻滞对妇科腹腔镜手术麻醉效果及术后镇痛的影响。方法选择2015年1月至2017年7月在上海市嘉定区南翔医院择期全身麻醉下行妇科腹腔镜手术患者60例,年龄46~68岁,美国麻醉医师协会(ASA)分级Ⅰ~Ⅱ级。采用随机数字表法,将其分为两组(n=30):对照组(A组)和超声引导下腹横肌平面阻滞组(B组)。B组于麻醉诱导前30min在超声引导下行双侧腹横肌平面阻滞。两组于手术结束前10min连接经静脉病人自检镇痛(PCIA)泵,持续镇痛48h,维持视觉模拟(VAS)评分<4分,当VAS评分≥4分时,静脉注射舒芬太尼5μg。记录两组术中麻醉药用量,苏醒及拔管时间,记录术后24h内舒芬太尼单位时间用量、镇痛补救情况和不良反应发生情况。结果与A组比较,B组术中丙泊酚用量[(210±32)mg比(324±45)mg]、舒芬太尼用量[(31±3.6)μg比(42±3.8)μg]、术后24h内舒芬太尼单位时间用量[(3.2±0.5)μg比(5.4±0.7)μg]明显减少(P<0.001);苏醒时间[(12±3)min比(20±5)min]及拔管时间[(15±4)min比(25±7)min]明显提前(P<0.001)。术后24h内镇痛补救率(13%比40%)和恶心(10%比33%)、呕吐(0%比10%)发生率降低(P<0.05)。结论超声引导下腹横肌平面阻滞,可明显减少妇科腹腔镜下全子宫切除手术患者术中麻醉药用量,改善术后早期镇痛效果。 展开更多
关键词 麻醉和镇痛 腹肌 神经肌肉阻滞 腹腔镜检查 妇科手术
Spinal and supraspinal control of motor function during maximal eccentric muscle contraction:Effects of resistance training
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作者 Per Aagaard 《运动与健康科学:英文版》 SCIE 2018年第3期282-293,共12页
Neuromuscular activity is suppressed during maximal eccentric(ECC)muscle contraction in untrained subjects owing to attenuated levels of central activation and reduced spinal motor neuron(MN)excitability indicated... Neuromuscular activity is suppressed during maximal eccentric(ECC)muscle contraction in untrained subjects owing to attenuated levels of central activation and reduced spinal motor neuron(MN)excitability indicated by reduced electromyography signal amplitude,diminished evoked H-reflex responses,increased autogenic MN inhibition,and decreased excitability in descending corticospinal motor pathways.Maximum ECC muscle force recorded during maximal voluntary contraction can be increased by superimposed electrical muscle stimulation only in untrained individuals and not in trained strength athletes,indicating that the suppression in MN activation is modifiable by resistance training.In support of this notion,maximum ECC muscle strength can be increased by use of heavy-load resistance training owing to a removed or diminished suppression in neuromuscular activity.Prolonged(weeks to months)of heavy-load resistance training results in increased H-reflex and V-wave responses during maximal ECC muscle actions along with marked gains in maximal ECC muscle strength,indicating increased excitability of spinal MNs,decreased presynaptic and/or postsynaptic MN inhibition,and elevated descending motor drive.Notably,the use of supramaximal ECC resistance training can lead to selectively elevated V-wave responses during maximal ECC contraction,demonstrating that adaptive changes in spinal circuitry function and/or gains in descending motor drive can be achieved during maximal ECC contraction in response to heavy-load resistance training. 展开更多
关键词 CORTICOSPINAL EXCITABILITY ECCENTRIC muscle CONTRACTION H-reflex NEUROMUSCULAR plasticity Resistance training V-wave
神经肌肉电刺激对低O2高CO2大鼠下肢骨骼肌miR-1相关通路的影响 预览
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作者 潘录录 支英豪 王小同 《温州医科大学学报》 CAS 2018年第2期86-90,共5页
目的:探讨神经肌肉电刺激(NMES)对慢性低O2高CO2大鼠腓肠肌的作用,并研究其对miR-1及相关蛋白通路的影响。方法:成年SD大鼠24只随机分成3组:对照组(NC组)、造模组(HH组)和电剌激组(HE组),每组8只。HH组和HE组大鼠置于低O2... 目的:探讨神经肌肉电刺激(NMES)对慢性低O2高CO2大鼠腓肠肌的作用,并研究其对miR-1及相关蛋白通路的影响。方法:成年SD大鼠24只随机分成3组:对照组(NC组)、造模组(HH组)和电剌激组(HE组),每组8只。HH组和HE组大鼠置于低O2高CO2舱内造模,每天持续8h,共4周。HF组在持续造模2周后,用电刺激仪对大鼠进行30min。2Hz和100Hz,1:1交替循环模式的NMES,维持2周:NC组仅做捆绑及贴电极片处理。动物跑台测定大鼠耐力运动能力,免疫组织化学观察腓肠肌肌纤维类型转变,qRT—PCR检测miR-1水平,Western blot检测腓肠肌核内组蛋白脱乙酰基酶4(HDAC4)、肌细胞促进因子2(MEF-2)和过氧化体增殖物激活型受体1,共激活因子1α(PGC-1α)蛋白含量的变化。结果:与NC组比,HH组大鼠腓肠肌慢肌纤维比例明显下降,miR-1含量明显升高,HDAC4、MEF2及PGC-1α蛋白含量明显下降,差异均有统计学意义(P〈0.05);大鼠耐力运动能力差异无统计学意义(P=0.054);与HH组比,HE组大鼠腓肠肌慢肌纤维比例明显升高,而miR-1含量下降,对应各蛋白含量均出现不同程度升高,大鼠耐力运动能力也得到了改善,差异均有统计学意义(P〈0.05)。结论:慢性低O2高CO2可致大鼠腓肠肌慢肌纤维向快肌纤维转变,从而导致运动耐力下降,而NMES可部分逆转这种病理变化,改善大鼠耐力运动能力。miR-1-HDAC4-MEF2-PGCg信号通路可能参与其中。 展开更多
关键词 低氧高二氧化碳 慢性阻塞性肺疾病 神经肌肉电刺激 组蛋白脱乙酰基酶4 MIR-1 大鼠
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神经肌肉与生物力学视角下标枪项目特征研究 预览
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作者 王立冬 曲淑华 李春光 《山东体育学院学报》 CSSCI 北大核心 2018年第5期100-105,共6页
基于神经肌肉以及生物力学视角对标枪运动项目特征进行论述,提出神经肌肉系统调控下的速度力量练习对于标枪运动员的重要性,从生物力学的角度强调了正确的用力顺序、身体各环节速度节奏对于技术训练的必要性。对标枪项目所需的专项力量... 基于神经肌肉以及生物力学视角对标枪运动项目特征进行论述,提出神经肌肉系统调控下的速度力量练习对于标枪运动员的重要性,从生物力学的角度强调了正确的用力顺序、身体各环节速度节奏对于技术训练的必要性。对标枪项目所需的专项力量、“冲量步”、左右腿技术动作等重要环节加以阐述,旨在增强广大教练以及运动员对这一项目特征本质的认识,提高训练的科学性。 展开更多
关键词 标枪 神经肌肉 运动生物力学 技术 项目特征
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Steroids and plasma exchange in Isaacs' syndromewith anti-Caspr2 antibodies
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作者 Daniele Orsucci Gianfranco Cafforio +3 位作者 Gianluca Moscato Alessandro Napolitano Marco Margelli Monica Mazzoni 《神经免疫与神经炎症(英文版)》 2018年第3期5-9,共5页
Isaacs' syndrome is a disease characterized by nerve hyperexcitability. The patients are commonly treated with symptomatic therapies and immunomodulatory approaches, but no clinical trials are available to date. H... Isaacs' syndrome is a disease characterized by nerve hyperexcitability. The patients are commonly treated with symptomatic therapies and immunomodulatory approaches, but no clinical trials are available to date. Here, we report the case of an anti-Caspr2-positive patient, presenting with continuous muscle twitches and diffuse muscle pain. He experienced a nearly complete clinical response to intravenous high-dose steroids combined with plasma exchange, sustained for at least 1 year. Our experience suggests that methylprednisolone 1000 mg/day × 5 days and consecutive tapering followed by plasma exchange may be efficient and well tolerated in patient with Isaacs' syndrome due to anti-Caspr2 antibodies. 展开更多
关键词 Contactin-associated protein-2 ISAAC NEUROMUSCULAR HYPEREXCITABILITY NEUROMYOTONIA VOLTAGE-GATED potassium channel
Predictors of residual force enhancement in voluntary contractions of elbow flexors
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作者 Heiliane de Brito Fontana Daiani de Campos Raphael Luiz Sakugawa 《运动与健康科学:英文版》 SCIE 2018年第3期318-325,共8页
Background:The steady-state increase in muscle force generating potential following a lengthening contraction is called residual force enhancement(RFE).In this study,we aimed to test for differences in torque,elect... Background:The steady-state increase in muscle force generating potential following a lengthening contraction is called residual force enhancement(RFE).In this study,we aimed to test for differences in torque,electromyographic activity(EMG),and the associated neuromuscular efficiency(NME)between isometric voluntary contractions of elbow flexors preceded and not preceded by a lengthening contraction.The dependence of such differences on(i)stretch amplitude,(ii)the region of the force-length(FxL)relationship where contraction occurs,and(iii)the individual's ability to produce(negative)work during the stretch was investigated.Methods:Sixteen healthy adults participated in the study.Elbow flexor torque,angle,and biceps brachii EMG for purely isometric contractions(reference contractions)and for isometric contractions preceded by active stretches of 20°and 40°were measured at the ascending,plateau,and descending regions of subject-specific FxL curves.All contractions were performed in an isokinetic dynamometer.Two-factor(stretch×FxL region)repeated measures analysis of variance ANOVAs was used to analyze the effect of active stretch on EMG,torque,and NME across conditions.The relationships between mechanical work during stretch-calculated as the torque-angular displacement integral-and the changes in EMG,torque,and NME were analyzed using Pearson correlation.Results:In general,torque,EMG,and NME following active stretches differed from the values observed for the purely isometric reference contractions.While although the detailed effects of active stretch on torque and EMG differed between regions of the FxL relationship,NME increased by about 19%for all muscle lengths.Up to 30%of the interindividual variability in torque generating potential change in response to active stretching was accounted for by differences in(negative)work capacity between subjects.Conclusion:Our results suggest that(i)RFE contributes to"flatten"the elbow flexor torque-angle rela 展开更多
关键词 Force-length History-dependent properties NEUROMUSCULAR efficiency UPPER LIMB VOLUNTARY CONTRACTIONS
Prediction of post-progression survival in patientswith advanced hepatocellular carcinoma treated with sorafenib by using time-dependent changes inclinical characteristics
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作者 Yoshiyuki Wada Yuko Takami +4 位作者 Hajime Matsushima Masaki Tateishi Tomoki Ryu Munehiro Yoshitomi Hideki Saitsu 《肝癌研究(英文版)》 2018年第3期1-8,共8页
Aim: Sorafenib has been shown to improve time to tumor progression (TTP) and overall survival (OS) in patients with hepatocellular carcinoma (HCC);however, post-progression survival (PPS) has not been well characteriz... Aim: Sorafenib has been shown to improve time to tumor progression (TTP) and overall survival (OS) in patients with hepatocellular carcinoma (HCC);however, post-progression survival (PPS) has not been well characterized in these patients. This study aimed to evaluate the predictors of PPS by using time-dependent and dynamic changes in radiologic progression patterns, liver function, and performance status (PS) in patients with advanced HCC receiving sorafenib treatment. Methods: We retrospectively analyzed the clinical characteristics of 128 advanced HCC patients with Child-Pugh scores ≤ 7 at the initiation of sorafenib treatment. Results: The median TTP, OS, and PPS were 3.8, 15.6, and 9.9 months, respectively. At the time of confirmation of radiologic progressive disease (PD), a total of 46 (35.6%) patients showed impairments in their PS of ≥ +1 points over time. For the Child-Pugh score, 27 (21.1%) and 26 (10.9%) patients exhibited an impairment of ≥ +1 and ≥ +2 points, respectively. Multivariate analysis identified the following independent predictors of PPS: impairment in the PS score of ≥+1 point [hazard ratio (HR) 1.81, 95% confidence interval (CI) 1.16-2.82], impairment in the Child-Pugh score of ≥ +2 points (HR 3.70, 95% CI 1.68-8.15), radiologic pattern of progression (target lesion growth and emergence of a new lesion) (HR 2.91, 95% CI 1.79-2.91), a TTP < 4 months(HR 1.87, 95% CI 1.21-2.91), second-line treatment after radiologic confirmation of PD (HR 0.16, 95% CI 0.08-0.32), and continuous sorafenib treatment after radiologic confirmation of PD (HR 1.76, 95% CI 1.06-3.00). Conclusion: PPS in patients with advanced HCC can be characterized by using time-dependent dynamic changes in clinical parameters. 展开更多
关键词 Contactin-associated protein-2 ISAAC NEUROMUSCULAR HYPEREXCITABILITY NEUROMYOTONIA VOLTAGE-GATED potassium channel
Practical approach to the patient with acute neuromuscular weakness 预览
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作者 Rajeev Nayak 《世界临床病例杂志》 2017年第7期270-279,共10页
Acute neuromuscular paralysis(ANMP)is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks(less than 4 wk).Bulbar and respiratory muscle weaknes... Acute neuromuscular paralysis(ANMP)is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks(less than 4 wk).Bulbar and respiratory muscle weakness may or may not be present.It is a common neurological emergency which requires immediate and careful investigations to determine the etiology because accurate diagnosis has significant impact on therapy and prognosis.Respiratory failure caused by neuromuscular weakness is considered as more critical than lung disease because its development may be insidious or subtle until sudden decompensation leads to life threatening hypoxia.Also,the arterial blood gas finding of severe hypoxemia,hypercapnia,and acidosis may not be apparent until respiratory failure is profound.Hence,the requirement for respiratory assistance should also be intensively and promptly investigated in all patients with neuromuscular disease.The disorder is classified based on the site of defect in motor unit pathway,i.e.,anterior horn cells,nerve root,peripheral nerve,neuromuscular junction or muscle.Identification of the cause is primarily based on a good medical history and detailed clinical examination supplemented with neurophysiologic investigations and sometimes few specific laboratory tests.Medical history and neurological examination should be focused on the onset,progression,pattern and severity of muscle weakness as well as cranial nerves testing and tests for autonomic dysfunction.Associated non neurological features like fever,rash or other skin lesions etc.should also be noted.Globally,Guillain-Barrésyndrome is the most frequent cause of ANMP and accounts for the majority of cases of respiratory muscles weakness associated with neuromuscular disorders.Newly acquired neuromuscular weakness in intensive care unit patients consist of critical illness polyneuropathy,critical illness myopathy and drug induced neuromuscular weakness which may arise as a consequence of sepsis,multi-organ failure,and exposure to certain medica 展开更多
关键词 NEUROMUSCULAR WEAKNESS PARALYSIS APPROACH NERVE MUSCLE
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Newborn screening for Duchenne muscular dystrophy in China: follow-up diagnosis and subsequent treatment
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作者 Qing Ke Zheng-Yan Zhao +11 位作者 Robert Griggs Veronica Wiley Anne Connolly Jennifer Kwon Ming Qi Daniel Sheehan Emma Ciafaloni R Rodney Howell Petra Furu Peter Sazani Arvind Narayana Michele Gatheridge 《世界儿科杂志(英文版)》 2017年第3期197-201,共5页
Background:Newborn screening for Duchenne muscular dystrophy (DMD) is currently being initiated in Zhejiang Province,China and is under consideration in other countries,including the United States.As China begins to i... Background:Newborn screening for Duchenne muscular dystrophy (DMD) is currently being initiated in Zhejiang Province,China and is under consideration in other countries,including the United States.As China begins to implement DMD newborn screening (DMD-NBS),there is ongoing discussion regarding the steps forward for follow up care of positively identified patients as well as false positive and false negative results.Data sources:Relevant papers related to DMD-NBS,and NBS in China were reviewed in PubMed.Results:The current state of DMD-NBS is discussed,along with the steps needed to effectively screen infants for this disease in China,recommendations for establishment of follow up care in patients with positive and negative screens,and measurement of patient outcomes.Conclusions:Zhejiang Province,China is ready to implement DMD-NBS.Future challenges that exist for this program,and other countries,include the ability to track patients,assist with access to care,and ensure adequate follow-up care according to evidence-based guidelines.In addition,China's large rural population,lack of specialty providers,and difficulty in educating patients regarding the benefits of treatment create challenges that will need to be addressed. 展开更多
关键词 Duchene MUSCULAR DYSTROPHY NEUROLOGY NEUROMUSCULAR disorders NEWBORN screening
Neuromuscular electrical stimulation and testosterone did not influence heterotopic ossification size after spinal cord injury:A case series 预览
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作者 Pamela D Moore Ashraf S Gorgey +4 位作者 Rodney C Wade Refka E Khalil Timothy D Lavis Rehan Khan Robert A Adler 《世界临床病例杂志》 2016年第7期172-176,共5页
Neuromuscular electrical stimulation(NMES)and testosterone replacement therapy(TRT)are effective rehabilitation strategies to attenuate muscle atrophy and evoke hypertrophy in persons with spinal cord injury(SCI).Howe... Neuromuscular electrical stimulation(NMES)and testosterone replacement therapy(TRT)are effective rehabilitation strategies to attenuate muscle atrophy and evoke hypertrophy in persons with spinal cord injury(SCI).However both interventions might increase heterotopic ossification(HO)size in SCI patients.We present the results of two men with chronic traumatic motor complete SCI who also had pre-existing HO and participated in a study investigating the effects of TRT or TRT plus NMES resistance training(RT)on body composition.The 49-year-old male,Subject A,has unilateral HO in his right thigh.The 31-year-old male,Subject B,has bilateral HO in both thighs.Both participants wore transdermal testosterone patches(4-6 mg/d)daily for 16 wk.Subject A also underwent progressive NMES-RT twice weekly for 16 wk.Magnetic resonance imaging scans were acquired prior to and post intervention.Cross-sectional areas(CSA)of the whole thigh and knee extensor skeletal muscles,femoral bone,and HO were measured.In Subject A(NMES-RT+TRT),the whole thigh skeletal muscle CSA increased by 10%,the knee extensor CSA increased by 17%,and the HO+femoral bone CSA did not change.In Subject B(TRT),the whole thigh skeletal muscle CSA increased by 13%in the right thigh and 6%in the left thigh.The knee extensor CSA increased by 7%in the right thigh and did not change in the left thigh.The femoral bone and HO CSAs in both thighs did not change.Both the TRT and NMES-RT+TRT protocols evoked muscle hypertrophy without stimulating the growth of preexisting HO. 展开更多
关键词 HETEROTOPIC OSSIFICATION NEUROMUSCULAR electrical stimulation TESTOSTERONE Resistance training Spinal cord injury
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前交叉韧带重建术后膝关节的神经肌肉功能重塑 预览 被引量:10
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作者 郭韵 杜良杰 +6 位作者 李建军 杨明亮 李军 冯雨桐 唐丽 刘长彬 高峰 《中国康复理论与实践》 CSCD 北大核心 2016年第1期65-68,共4页
前交叉韧带损伤或重建术后,膝关节本体感觉改变,中枢神经功能重塑,运动控制功能缺陷。需对膝关节进行系统的神经肌肉功能重塑,包括本体感觉恢复、中枢神经系统重塑和强化运动控制。
关键词 前交叉韧带重建术 本体感觉 运动控制 神经肌肉 功能重塑 综述
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意拳训练介入振动刺激对下肢肌肉影响的研究
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作者 李臣 郑勤 宋彩珍 《西安体育学院学报》 CSSCI 北大核心 2016年第1期94-100,共7页
目的探讨意拳训练时接受振动刺激对下肢肌肉及其神经支配的影响。方法 48位健康大学生分为3组:意拳振动组(YQV)、意拳组(YQ)以及控制组(CON),进行为期8周、每周3次的训练。训练前后以Biodex System 4等速训练系统、Biopac多导生... 目的探讨意拳训练时接受振动刺激对下肢肌肉及其神经支配的影响。方法 48位健康大学生分为3组:意拳振动组(YQV)、意拳组(YQ)以及控制组(CON),进行为期8周、每周3次的训练。训练前后以Biodex System 4等速训练系统、Biopac多导生理记录仪及AMTI三维测力台,测试下肢最大等长肌力、等速肌力、肌电反应及静态平衡。统计分析以双因素方差分析进行。结果意拳组的60°/s等速向心肌力显著进步11.8%(P〈0.05);静态平衡时,足底压力中心移动面积显著减少15.3%(P〈0.05),并明显少于控制组(P〈0.05);意拳组在静态平衡的进步率(9.3%)显著优于控制组(P〈0.05),其余测试项则无差异;控制组在各因变量中无显著变化。结论意拳结合振动刺激进行训练能有效增进膝伸肌等速肌力与下肢静态平衡的表现,可作为促进下肢肌肉及神经支配表现的新式训练方法。 展开更多
关键词 意拳 振动刺激 下肢肌肉 神经肌肉
Efficacy of different doses of sugammadex after continuous infusion of rocuronium 预览
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作者 Diego Soto Mesa Mounir Fayad Fayad +5 位作者 Laura Pérez Arviza Verónica Del Valle Ruiz Fernando Cosio Carreno Luis Arguelles Tamargo Manuel Amorín Díaz Sergio Fernández-Pello Montes 《世界临床病例杂志》 2015年第4期360-367,共8页
AIM:To evaluate the effects of two different doses of sugammadex after maintenance anesthesia with sevofluorane and remifentanil and deep rocuroniuminduced neuromuscular blockade(NMB).METHODS:Patients between 20 and 6... AIM:To evaluate the effects of two different doses of sugammadex after maintenance anesthesia with sevofluorane and remifentanil and deep rocuroniuminduced neuromuscular blockade(NMB).METHODS:Patients between 20 and 65 years of age,with American Society of Anesthesiologists physical status classificationⅠ-Ⅱ,undergoing gynecological surgery were included in a prospective,comparative and randomized study.NMB was induced with an injection of 0.6 mg/kg of rocuronium followed by continuous infusion of 0.3-0.6 mg/kg per hour to maintain a deep block.Anesthesia was maintained with sevofluorane and remifentanil.Finally,when surgery was finished,a bolus of 2 mg/kg(group A)or 4 mg/kg(group B)of sugammadex was applied when the NMB first response in the train-of-four was reached.The primary clinical endpoint was time to recovery to a train-of-four ratio of 0.9.Other variables recorded were the time until recovery of train-of-four ratio of 0.7,0.8,hemodynamic variables(arterial blood pressure and heart rate at baseline,starting sugammadex,and minutes 2,5 and 10)and adverse events were presented after one hour in the post-anesthesia care unit.RESULTS:Thirty-two patients were included in the study:16 patients in group A and 16 patients in group B.Only 14 patients each group were recorded because arterial pressure values were lost in two patients from each group in minute 10.The two groups were comparable.Median recovery time from starting of sugammadex administration to a train-of-four ratio of 0.9 in group A and B was 129 and 110 s,respectively.The estimated difference in recovery time between groups was 24 s(95%CI:0 to 45 s,Hodges-Lehmann estimator),entirely within the predefined equivalence interval.Times to recovery to train-of-four ratios of 0.8(group A:101 s;group B:82.5 s)and 0.7(group A:90 s;group B:65 s)from start of sugammadex administration were not equivalent between groups.There was not a significant variation in the arterial pressure and heart rate values between the two groups and none of the patients showed 展开更多
关键词 ROCURONIUM SUGAMMADEX NEUROMUSCULAR BLOCK ANTAGONISM Monitoring NEUROMUSCULAR function NEUROMUSCULAR BLOCK ROCURONIUM
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Neuromuscular control and rehabilitation of the unstable ankle 预览
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作者 You-jou Hung 《世界骨科杂志》 2015年第5期434-438,共5页
Lateral ankle sprain is a common orthopedic injury with a very high recurrence rate in athletes.After decades of research,it is still unclear what contributes to the high recurrence rate of ankle sprain,and what is th... Lateral ankle sprain is a common orthopedic injury with a very high recurrence rate in athletes.After decades of research,it is still unclear what contributes to the high recurrence rate of ankle sprain,and what is the most effective intervention to reduce the incident of initial and recurrent injuries.In addition,clinicians often implement balance training as part of the rehabilitation protocol in hopes of enhancing the neuromuscular control and proprioception of the ankle joint.However,there is no consensus on whether the neuromuscular control and proprioception are compromised in unstable ankles.To reduce the prevalence of ankle sprains,the effectiveness of engaging balance training to enhance the neuromuscular control and proprioception of the ankle joint is also questionable. 展开更多
关键词 ANKLE PROPRIOCEPTION NEUROMUSCULAR control PHYSICAL therapy REHABILITATION
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