单纤维肌电图诊断眼肌型重症肌无力的敏感性分析

目的分析眼肌型重症肌无力(OMG)患者额肌单纤维肌电图(SFEMG)表现并辅助临床诊断。方法选择临床确诊为OMG患者27例,对患者行额肌SFEMG检测,同时行低频重复神经电刺激(RNS)、乙酰胆碱受体抗体(AChR-Ab)测定和新斯的明试验,比较上述检查方法的敏感性差异。结果额肌SFEMG检查结果显示,有18例患者(66.7%)异常,表现为颤抖增宽,其中6例伴有阻滞,平均颤抖值为(45.4±21.0)μs,高于正常对照组;颤抖值>55μs的电位对百分比为0%~80.0%,平均(24.2±24.5)%;阻滞百分比为0%~14%,平均(1.90±4.40)%;AchR-Ab滴度增高者8例(29.6%),其诊断OMG敏感性与额肌SFEMG敏感性存在差异;RNS异常者9例(33.3%),新斯的明试验阳性者23例(85.2%),上述2种检测方法与额肌SFEMG检查敏感性差异无统计学意义。结论额肌SFEMG检测是诊断OMG的一种敏感性较高的检测方法。     

Single-fiber Electromyography in the Extensor Digitorum Communis for the Predictive Prognosis of Ocular Myasthenia Gravis: A Retrospective Study of 102 Cases

Background:

Single-fiber electromyography (SFEMG) abnormality in the extensor digitorum communis (EDC) was reported in ocular myasthenia gravis (OMG), which indicated subclinical involvement beyond extraocular muscles in OMG patients. The relationship between the abnormal findings of SFEMG in EDC and the probability for OMG to develop generalized myasthenia gravis (GMG) is unknown. This retrospective study aimed to determine the predictive value of abnormality of SFEMG in EDC of OMG patients.

Methods:

One-hundred and two OMG patients underwent standard clinical diagnosis process and SFEMG test in EDC muscle when diagnosed and were clinically followed up for 5 years. The SFEMG data were compared between different clinical groups according to thymus status, onset age, and different outcome of OMG developing. Chances of progressing to GMG were compared between two different groups according to SFEMG and repetitive nerve stimulation (RNS) results, acetylcholine receptor antibody (AchRAb) titer, thymus status, and onset age.

Results:

Abnormal SFEMG results were observed in 84 (82.4%) patients. The mean jitter, percentage of jitter >55 μs (%), and blocking were higher in OMG patients than in healthy volunteers. There were no statistical differences in jitter analysis between thymoma group and non-thymoma group (P = 0.65), or between the later OMG group and the later GMG group (P = 0.31), including mean jitter, percentage of jitter >55 μs (%), and blocking. Elderly group (≥45 years old) had a higher mean jitter than younger group (t = 2.235, P = 0.028). Total 55 OMG developed GMG, including 47 in abnormal SFEMG group while 8 in normal SFEMG group. There was no statistical difference in the conversion rates between the two groups (χ² = 0.790, P = 0.140). RNS abnormality, AchRab titer, or onset age had no correlation with OMG prognosis (P = 0.150, 0.070, 0.120, respectively) while thymoma did (χ² = 0.510, P = 0.020).

Conclusion:

SFEMG test in the EDC showed high abnormality in OMG, suggesting subclinical involvement other than extraocular muscles. Nevertheless, the abnormal jitter analysis did not predict the prognosis of OMG according to clinical follow-up.     

Single fiber electromyography in 78 patients with amyotrophic lateral sclerosis

Background Single fiber electromyography (SFEMG) is a sensitive technique for detecting abnormalities in neuromuscular transmission and is mainly used in the diagnosis of neuromuscular junction disorders, such as myasthenia gravis. While the process of denervation-reinnervation in amyotrophic lateral sclerosis (ALS) can also result in immature collateral nerve terminals and instability of neuromuscular transmission, the purpose of this study was to investigate the changes and clinical values of SFEMG in patients with ALS.Methods Volitional SFEMG was performed on the extensor digitorum communis (EDC) of 78 patients with ALS (men 52, women 26) who had been previously diagnosed by history, clinical features, and neurophysiological studies. The mean jitter, the percentage of jitter >55 μs, the impulse blocking percentage, and fiber density (FD) were determined. These results were compared to normal controls. In addition, the SFEMG indices were analyzed for correlations with the duration of ALS, the EDC strength score on the Medical Research Council (MRC) scale, and spontaneous activity detected by EMG studies. Results SFEMG indices were abnormal in all patients with ALS. Mean jitter ranged from 30 to 178 μs (mean 80.2 μs); the percentage of jitter >55 μs ranged from 5% to 100% (mean 60.5%). In addition, the impulse blocking percentage ranged from 0% to 90% (mean 28.1%) and FD ranged from 1.4 to 4.1 (mean 2.6). Mean jitter, the percentage of jitter >55 μs, and the blocking percentage in 57 patients with definite or probable ALS were significantly higher than in patients with possible or suspected ALS. MRC scores of the EDC negatively correlated with mean jitter, the percentage of jitter >55 μs, blocking percentage, and FD. Conclusions SFEMG is the most sensitive tool for diagnosing definite or probable ALS. Increased jitter, blocking percentage, and FD can indicate the degree of immature collateral sprouts and motor end plates resulting from the progressive denervation and reinnervation associated with ALS, and may be helpful in evaluating prognosis.     

CHANGES OF SINGLE FIBER ELECTROMYOGRAPHY IN PATIENTS WITH INFLAMMATORY MYOPATHIES

Objective To assess the significance of single fiber electromyography (SFEMG) in diagnosis of inflammatory myopathies and the correlation with other assistant examination findings.Methods SFEMG were recorded from the extensor digitorum communis of 34 patients with polymyositis or dermatomyositis and compared with the fmdings of routine electromyography (EMG), serum creatine kinase (CK) determination,and muscle biopsy.Results SFEMG recordings in 34 patients were all abnormal. The prominent feature was markedly increased fiber density (FD) with normally or mildly increased jitter. FD ranged from 1.0 to 6.0 (2.34±0.43). Jitter ranged from 5 to 78μs (41.6±10.3 μs). The potential pairs with jitter values greater than 55 μs ranged from 0% to 55% (7.7% ± 11.8%). Block was detected at one recording site in only one patient. Routine EMG demonstrated myogenic lesions in only 24 patients (70.6%). FD was a little higher in the normal-EMG subgroup or the neurogenic-EMG subgroup than myogenic-EMG subgroup but without statistical significance. Elevated CK levels were found in 75% patients (24/32). FD in the normal CK subgroup was significantly higher than that in the elevated CK subgroup (2.62±0.40 vs. 2.28±0.40, P ＜ 0.05). Muscle pathologies were consistent with the diagnosis of myositis in 75% (15/20).Conclusion SFEMG is of great value in the diagnosis and disease process understanding of inflammatory myopathies for the clinically suspected patients with normal routine EMG, CK levels, and muscle biopsies.     

重症肌无力伴胸腺瘤或胸腺增生患者神经电生理特点及手术的影响

目的探讨重症肌无力伴胸腺瘤及胸腺增生患者的电生理指标特点,并观察胸腺切除手术对于电生理指标的影响。方法对12例伴有胸腺瘤、25例伴有胸腺增生的重症肌无力患者进行伸指总肌单纤维肌电图（SFEMG）、重复神经电刺激（RNS）和肌电图（EMG）检测,比较两组间的神经电生理特点,同时观察手术前、后电生理指标的变化。结果 12例伴有胸腺瘤患者术前的SFEMG、RNS、肌电图的异常率分别为100%、41.4%和25%,25例伴有胸腺增生患者术前的SFEMG、RNS、肌电图异常率分别为92%、52%和32%;12例伴有胸腺瘤患者手术后的SFEMG、RNS、肌电图异常率为91.7%、33.3%和25%,25例胸腺增生手术后为86.9%、40%和24%。结论伴有胸腺瘤及胸腺增生患者的SFEMG阳性率最高,其次为RNS、肌电图,手术后电生理指标有改善,而其中胸腺增生者改善更为明显,但均仍存在异常,仍需药物治疗。     

单纤维肌电图检测运动神经元病患者临床研究

目的 探讨单纤维肌电图检测运动神经元病(MND)的临床意义。方法 用单纤维肌电图对38例MND患者进行颤抖 (用MCD表示 )和肌纤维密度(FD)测定,同时进行常规肌电图检查。结果 MND组较对照组颤抖明显增宽 ,FD增加 (P<0.001) ;病程与MCD值和FD值无相关性 ;单纤维肌电图对MND的检出率(92.11%)大于常规肌电图(76.32 % ,精确概率法,P<0.05)。 结论 MND存在神经肌肉接头传递功能障碍和“失神经—神经再支配”现象 ;MND病程与MCD值、FD值无显著相关性 ;对MND的检出单纤维肌电图较常规肌电图敏感。     

Jitter analysis with concentric needle electrodes in the extensor digitorum communis for the diagnosis of myasthenia gravis: a pilot study

Background Single-fiber electromyography is the most sensitive neurophysiological test for the diagnosis of myasthenia gravis （MG）,but its use is limited by the potential risk of transmission of infections.Jitter analysis with disposable concentric needle electrodes （CNEs） is therefore being investigated.This pilot study aimed to evaluate jitter analysis with CNEs for the diagnosis of MG.Methods Forty-two healthy Chinese volunteers and 44 MG patients were prospectively enrolled.MG patients were classified according to the Osserman classification,and acetylcholine receptor antibody titer was measured.Jitter analysis with CNEs in the extensor digitorum communis and repetitive nerve stimulation （RNS） testing were performed.Jitter was expressed as the mean consecutive difference （MCD）,and 20 action potential pairs were analyzed in each subject.The mean MCD in each subject and the mean individual MCD of all action potential pairs were compared between groups.Results The mean MCD and mean individual MCD were higher in MG patients （（42.3±20.0） μs and （42.2±26.0） μs） than in healthy volunteers （（23.0±3.1） μs and （22.8±7.5） μs）.The area under the receiver operating characteristic curve for the mean MCD of MG patients and healthy volunteers combined was 0.85.The mean MCD and mean individual MCDwere higher in generalized MG patients （（64.1±18.5） μs and （63.6±30.0） μs） than in ocular MG patients （（33.1±12.0） μs and （33.2±17.6） μs）,and were higher in MG patients with abnormal RNS results （（57.2±18.3） μs and （57.3±29.2） μs） than in MG patients with normal RNS results （（32.9±14.8） μs and （32.7±18.3） μs）.Abnormal RNS results were observed in 38.60% （17/44） of MG patients and abnormal jitter were observed in 72.70% （32/44） of MG patients.Conclusion Jitter analysis with CNEs is feasible for the diagnosis of MG.     

大剂量丙种球蛋白静注治疗重症肌无力的疗效研究

目的 探讨重症肌无力(MG)的免疫状态和临床效果及其在大剂量丙种球蛋白(HDIVIg)静注治疗前后的变化,并观察HDIVIg治疗的临床疗效。方法 用ELISA法检测36例MG患者治疗前后血清乙酰胆碱受体的抗体(AchRAb)和肿瘤坏死因子-α(TNF—α) 变化,并以20例健康者作为对照。比较HDIVIg治疗前后MG患者上述因子的变化。结果 患者治疗有效率为100％,复发率15％。治疗前后AchRAb、TNF-α均有明显的改变。结论 HDIVIg能够改善MG患者的兔疫功能,且显效快、复发率低。     

低频重复电刺激检查在重症肌无力诊断中的临床意义

目的 探讨低频重复电刺激(RNS)检查在重症肌无力(MG)患者中的诊断作用.方法 回顾性分析2008年7月至2010年8月住院确诊436例MG患者低频RNS的检查结果,分析MG患者中低频RNS阳性率,不同受榆肌群及MG各临床分型低频RNS异常率.结果 MG患者中低频RNS阳性率为73.85%.低频RNS异常以刺激面神经...     

大鼠电刺激单纤维肌电图的实验研究

目的建立大鼠的电刺激单纤维肌电图（S-SFEMG）引导方法，并观察各种影响因素对颤抖（jitter）值的影响。方法将健康成年sD大鼠用3％戊巴比妥钠（30mg／kg，腹腔注射）麻醉，暴露右侧坐骨神经及腓肠肌，采用丹迪Keypoint4M／4C型多功能肌电图仪测量腓肠肌jitter的平均连续差值（MCD）值及肌纤维密度（FD）值。分别改变刺激强度、刺激频率及体温观察测量结果变化。结果头测10只大鼠腓肠肌260个位点的MCD正常值范围为12—62us，均值为（32.5±10．5）us。温度、刺激频率对MCD值有影响，刺激强度改变对MCD值未见有明显影响。大鼠腓肠肌的FD值为（1.39±0．11）us。结论刺激及操作条件对电刺激单纤雏肌电图的检测结果有影响。SFEMG可客观、准确地反映外周神经纤维、神经．肌肉接头、肌纤维的状态，对基础医学及临床医学的研究具有重要的应用价值。     

重症肌无力重复神经电刺激检测的比较研究

目的比较肘肌、三角肌、小指展肌重复神经电刺激（RNS）对重症肌无力（MG）的诊断价值。方法应用低频RNS技术对18例MG患者的肘肌、三角肌、小指展肌进行疲劳前后的检测,用DANTECounterpoint肌电图仪记录。结果对照组肘肌、三角肌、小指展肌疲劳前RNS电压分别降低了1．54％±1．32％、1．64％±1．43％、0．62％±0．38％;疲劳后RNS电压分别降低了2．01％±1．75％、2．05％±1．98％、1．02％±0．75％。MG组肘肌、三角肌、小指展肌疲劳前测试阳性分别为9／18（50％）、10／18（55．6％）、3／18（16．7％）（P均＜0．05）;疲劳后RNS测试阳性分别为15／18（83．3％）、14／18（77．8％）、6／18（33．3％）（P均＜0．05）。经卡方检验,肘肌疲劳前后RNS测试阳性率有显著性差异（P＜0．05）。小指展肌、三角肌疲劳前后RNS测试阳性率对比无显著性差异。结论MG患者肘肌疲劳后RNS阳性率较疲劳前明显增高,而三角肌、小指展肌疲劳前后RNS阳性率无显著性差异;肘肌、三角肌RNS测试较小指展肌更敏感,但肘肌RNS方法简便,病人不适程度轻,有较大的临床实用价值。     

女性单纯声带肥厚和声带小结的嗓音参数分析比较

目的：通过比较和分析单纯声带肥厚和声带小结的嗓音参数的变化,评价这些参数在两种疾病中诊断及疗效。方法：应用Dr.Speech(DSS)软件对成人女性30例单纯声带肥厚和声带小结患者做噪音测试,对其主要声学参数进行比较分析。结果：声带小结的嗓音声学参数、基频微扰、声门噪声能量NEE值均较声厚的各指标高。其中声门噪声能量在声结中异常者占99．6％,声带肥厚占80％,两组之间有显著性差异（P＜0．05）。其频微扰和振幅微扰的异常占有率,两组之间无显著性差异（P＞0．05）。结论：嗓音参数对诊断和区别声带肥厚和声结有一定的价值,特别是声门噪声能量有更高的敏感性,对诊断和治疗声带疾病有一定的价值。     

重复神经电刺激在重症肌无力中的临床应用研究

目的　探讨重复神经电刺激 (RNS)对重症肌无力 (MG)的临床应用价值。方法　对 3 0例MG患者的面神经、副神经、腋神经、尺神经分别用低频 (3Hz、5Hz)和高频 (10Hz、15Hz、2 0Hz)超强电流进行RNS检查 ,共检查 12 0条神经。所检神经对应的肌肉疲劳后 ,再以上述频率和电流重复进行RNS检查。结果　低频刺激阳性为 5 6.67% (17/ 3 0 ) ,高频刺激阳性为 2 6.67% (8/ 3 0 ) ,两者比较差异有高度显著性 (P <0 .0 0 1)。 10 0 %RNS阳性患者在低频刺激时即可获得阳性结果。疲劳前的RNS阳性率为 5 6.67% (17/ 3 0 ) ,疲劳后为 83 .3 3 % (2 5 / 3 0 ) ,两者比较差异有显著性 (P <0 .0 5 )。疲劳后按 4块肌肉统计RNS阳性率为 86.67% (2 6/ 3 0 ) ,而按 3块肌肉统计阳性率为 60 .0 0 % (18/ 3 0 ) ,两者比较差异有显著性 (P <0 .0 5 )。结论　低频RNS检查在MG疾病诊断中具有重要的价值 ,对MG患者的临床分型和指导治疗具有重要意义 ,受检神经所支配的肌肉疲劳后和增加检查的神经数目能提高RNS阳性率。     

重症肌无力患者血清中乙酰胆碱受体抗体及肌肉特异性激酶抗体的检测及其意义

[目的]探讨重症肌无力（MG）患者血清中乙酰胆碱受体抗体（AchRAb）以及肌肉特异性激酶抗体（MuSKAb）与临床特点的关系．[方法]应用酶联免疫吸附试验（ELISA）,对40例MG患者血浆进行AchRAb和MuSKAb检测,并与患者临床特点进行比较分析．[结果]40例MG患者中AchRAb阳性为13例（33％）,MuSKAb阳性为7例（18％）,AchRAb与MuSKAb双阴性为22例（55％）,AchRAb与MuSKAb双阳性为2例（5％）．[结论]MG患者中AchRAb阳性者的病情普遍较轻,可受胸腺影响;MuSKAb阳性患者女性多见,全身症状严重．     

72例重症肌无力伴胸腺瘤的临床及神经电生理研究

目的探讨重症肌无力伴胸腺瘤（MGT）的临床和神经电生理特点。方法回顾性分析72例MGT和63例不伴胸腺瘤MG（NMGT）临床及神经重复电刺激（RNS）患者资料,数据应用SPSS13．0软件进行统计。结果MGT好发于35～59岁之间（72．2％）,平均发病年龄大于NMGT（39．5岁VS29．4岁）,平均病程短于NMGT（13．1个月VS29．1个月）。男女发病基本无差别（各占50％）。全身型比率高于NMGT（72．8％VS66．7％）。呼吸肌较NMGT易受累（20．8％VS9．5％）。病情进展快,自起病至呼吸肌和球肌受累平均时间均明显短于NMGT（12个月VS26．4个月;7个月VS11．6个月）。MGT的RNS检测有较高的阳性率,但与NMGT相比差异无统计学意义（86．9％VS75．0％）。97．2％（70例）可通过胸腺CT发现胸腺瘤或纵隔异常。乙酰胆碱受体抗体（AchRab）阳性率与NMGT差异无统计学意义（50．0％VS52．4％）。结论MGT多见于中老年,男女均好发。病程进展快,易有全身骨骼肌受累,呼吸肌受累比率高,神经电生理是MGT的敏感检查手段。AchRab可能不是检测MGT的特异抗体。胸腺CT是MGT术前重要的辅助检查手段。     

RNS技术在重症肌无力的应用

目的研究重复神经刺激技术(RNS)对重症肌无力(MG)疾病的诊断价值。方法分别对我院诊治的36例MG患的面神经、腋神经及尺神经进行RNS检查,共检查108条神经。结果108条神经中RNS阳性率为60．2％(65条),其中以腋神经对应的三角肌阳性率最高,为77．8％(28／36),尺神经对应的小指展肌阳性率最低,为41．7％(15／36)。同一患有一条或多条神经RNS阳性的病例数为总例数的86．1％(31／36)。所有RNS阳性的MG患在低频刺激即可获得阳性结果,最佳刺激频率为3～5Hz。结论对MG患同时进行腋神经、面神经和尺神经的重频刺激,可提高RNS的阳性率。     

慢性乙醇中毒性周围神经病大鼠的单纤维肌电图研究

目的观察单纤维肌电图（single fiber electromyography，SFEMG）对慢性乙醇中毒性周围神经病的早期诊断作用。方法建立慢性乙醇中毒性周围神经病大鼠模型，在实验开始后第8，12，16周对大鼠腓肠肌进行SFEMG检测，观察肌纤维密度（FD）、颤抖值（jitter）的变化。结果第8周，第12周SFEMG对乙醇组大鼠坐骨神经检测结果未见异常；第16周SFEMG检测结果显示乙醇组大鼠的MCD和FD值均增大，与对照组比较，差异有显著性（P〈0．05），并且出现电位对阻滞。结沦SFEMG可用于早期诊断慢性乙醇中毒性周围神经病，灵敏性较高.     

胸腺摘除对眼肌型重症肌无力疗效及影响因素分析

目的 探讨胸腺摘除术对眼肌型重症肌无力(OMG)疗效及影响因素分析.方法 回顾从1998年6月至2009年3月接受治疗的OMG患者135例,其中胸腺摘除术治疗65例,药物治疗70例.绘制生存曲线比较两种不同方式治疗OMG的转型率及复发率;采用COX比例风险回归模型对性别、年龄、术前病程、AChR Ab、Titin Ab、胸腺病理、术后激素治疗等影响因素进行分析,进一步比较Titin Ab水平、不同胸腺病理类型术后的复发率及转型率.结果 中位随访期为62个月.胸腺摘除治疗组总有效率72.3%,药物治疗组总有效率34.3%,差异有统计学意义(P<0.01).Titin Ab水平、胸腺病理及术后是否使用激素与术后疗效显著相关(均P<0.05).Titin Ab阳性及合并胸腺瘤的患者术后复发率及转型率明显高于Titin Ab阴性及不伴胸腺瘤患者(P<0.05).结论 胸腺摘除术是治疗OMG的有效手段,可改善症状,阻止复发及向全身型重症肌无力(GMG)转型.     

低频重复神经电刺激疲劳试验对重症肌无力患者的诊断作用

目的:对重症肌无力(MG)患者行低频重复神经电刺激(RNS)检测中的疲劳试验,阐明疲劳试验对MG患者的诊断价值。方法:收集30例MG患者,对面神经进行静息状态低频RNS检测,并进行疲劳试验,分别疲劳1和2 min,在疲劳结束后的即刻、休息1 min及2 min再进行RNS检测。分析静息及疲劳后不同时间点RNS中复合肌肉动作电位(CMAP)波幅递减百分率的差异。结果:低频RNS疲劳试验各时间点CMAP波幅递减幅度均较静息状态递减幅度明显,30例患者中以疲劳2 min后休息1 min RNS递减幅度最明显。30例患者中12例患者RNS静息状态下CMAP波幅未见明显递减现象,而疲劳后CMAP波幅递减明显。结论:低频RNS疲劳试验可明显提高MG患者的诊断率,尤其对于静息状态下RNS检测正常的MG患者有效。检测时以疲劳2 min后休息1 min效果最明显。     

颈内-后交通动脉瘤的介入治疗疗效分析

目的探讨颈内后交通动脉瘤血管内栓塞技术要点及疗效。方法对145例颈内后交通动脉瘤病人应用微导管技术,在数字减影血管造影(DSA)下行血管内栓塞治疗,其中对55例病人62个动脉瘤应用机械可脱性弹簧圈(MDS)栓塞,90例106个动脉瘤应用电解可脱性弹簧圈(GDC或EDC)栓塞。结果成功栓塞145例病人168个动脉瘤,其中动脉瘤达到100%栓塞的为96个,95%栓塞的为45个,90%栓塞的为15个,<90%栓塞的为12个。术中动脉瘤破裂3例,脑血管痉挛5例,脑梗死1例。118例病人痊愈,23例好转,2例恶化,3例死亡。通过DSA进行随访17例,3例3个动脉瘤复发,其中2个动脉瘤再次使用EDC达致密填塞。结论血管内栓塞治疗是颈内后交通动脉瘤安全、有效的治疗措施。