肯尼迪病的临床及肌电图分析

目的总结肯尼迪病的临床特征及肌电图表现,为早期诊断提供依据。方法回顾性分析2016年6月至2019年4月于中国医科大学附属盛京医院就诊的5例肯尼迪病患者的资料,所有患者均行肌电图检查。结果本组病例于就诊时均伴有肢体无力表现,但首发症状不尽相同。多数病例于就诊时临床症状主要表现为蹲起不能、上楼费力、行走困难、双上肢上举困难等。肌电图检查:4例患者出现感觉神经不同程度的波幅降低甚至不能引出,速度正常或轻度减慢。1例患者感觉神经传导速度及波幅正常。4例患者存在运动神经传导波幅降低,速度正常或轻度减慢。1例患者出现正中神经、胫神经F波出现率降低。5例患者四肢肌肉静止时可见少量自发电位,轻收缩及大力募集时存在广泛神经源性损害。肌电图检查时神经传导出现以感觉神经波幅受累为主,肌电图检查自发电位少,伴有肌肉神经源性损害时,结合相关临床表现,考虑肯尼迪病的可能。结论肌电图检查是一项方便、快捷的检查,对肯尼迪病可以起到很好的诊断及鉴别诊断的作用。     

慢性正己烷中毒神经损害的肌电图评价

目的 分析慢性正己烷中毒的神经肌电图表现。方法 用常规肌电图检查技术对16例慢性正己烷中毒患进行肌电图神经传导速度检测。结果 慢性正己烷中毒患电生理检查异常率为69％，感觉神经传导速度减慢为29.7%，运动神经传导速度减慢为59.4%，远端潜伏期延长为51.6%。结论 慢性正己烷中毒一损害运动神经远端为主，神经损害程度与接触时间长短有关。     

慢性正己烷中毒神经损害的肌电图评价

目的分析慢性正己烷中毒的神经肌电图表现。方法用常规肌电图检查技术对16例慢性正己烷中毒患者进行肌电图神经传导速度检测。结果慢性正己烷中毒患者电生理检查异常率为69%,感觉神经传导速度减慢为29.7%,运动神经传导速度减慢为59.4%,远端潜伏期延长为51.6%。结论慢性正己烷中毒一损害运动神经远端为主,神经损害程度与接触时间长短有关。     

低钾性麻痹与格林—巴利综合生临床和电生理对比研究

对４６例低钾性麻痹患者和９１例格林－巴利综合征（ＧＢＳ）患者进行了临床和电生理对比研究。结果表明：低钾麻痹组运动神经传导速度正常，运动诱发电位波幅降低。ＧＢＳ组又分为两种类型，即脱髓鞘型和运动轴索损害型，脱髓鞘型运动神经传导速度减慢，运动诱发电位波幅降低。而运动轴索损害型运动神经传导速度正常，运动诱发电位波幅亦降低，一波幅恢复时间不同于低钾性麻痹。     

低钾性麻痹与格林-巴利综合征临床和电生理对比研究

对46例低钾性麻痹患者和91例格林-巴利综合征（GBS）患者进行了临床和电生理对比研究。结果表明：低钾麻痹组运动神经传导速度正常，运动诱发电位波幅降低。GBS组又分为两种类型，即脱髓鞘型和运动轴索损害型。脱髓鞘型运动神经传导速度减慢，运动诱发电位波幅降低．而运动轴索损害型运动神经传导速度正常，运动诱发电位波幅亦降低，但病后波幅恢复时间不同于低钾性麻痹。     

糖尿病周围神经病变的电生理检测

目的：探讨电生理检测对糖尿病周围神经病的诊断意义。方法：对100例糖尿病患测定正中神经肘→腕运动神经传导速度、波幅，指1→腕感觉神经传导速度、波幅。迟神经肘→腕运动神经传导速度、波幅，指5→腕感觉神经传导速度、波幅。胫后神经Guo→踝运动神经传导速度、波幅，趾1→踝感觉神经传导速度、波幅。腓总神经膝→踝运动神经传导速度、波幅。腓肠神经外踝→腓肠点感觉神经传导速度及波幅。结果：糖尿病周围神经病以轴索及髓鞘同时受损多见，在四肢各神经中，感觉神经传导速度减慢及波幅降低较多，复合肌肉动作电位波幅降低次之，而运动神经传导速度受累较少见。结论：神经电生理检查对糖尿病周围神经病诊断具有重要意义。     

神经电图与肌电图评估肌萎缩侧索硬化症与脊髓型颈椎病患者的神经电生理差异

背景：肌萎缩侧索硬化症早期症状往往局限于某部位，与脊髓型颈椎病临床表现极其相似，但两者的治疗方法和预后截然不同。此时肌电图和神经电图的多部位检查具有重要参考价值，特别是胸段棘旁肌肌电图可作为区别肌萎缩侧索硬化症与脊髓型颈椎病的客观指标。目的：探讨肌萎缩侧索硬化症与脊髓型颈椎病的电生理改变差异。设计：回顾性病例分析。单位：江西医学院第二附属医院的神经内科。对象：选择2001—12／2004—11江西医学院第二附属医院神经内科门诊和住院的肌萎缩侧索硬化症30例患者和脊髓型颈椎病30例患者。方法：对肌萎缩侧索硬化症患者30例和脊髓型颈椎病30例进行常规肌电图、神经电图检测。肌电图检测包括三肢体肌＋胸锁乳突肌＋胸段棘旁肌，观察静息状态时自发电位，测定运动单位电位的时限、波幅，大力收缩时的募集相。神经电图测定运动传导速度和感觉传导速度及动作电位的末端潜伏期、波幅。主要观察指标：①肌萎缩侧索硬化症与脊髓型颈椎病患者肢体肌、胸锁乳突肌与棘旁肌的肌电图检测结果。②肌萎缩侧索硬化症与脊髓型颈椎病患者神经传导速度检测结果。结果：60例患者全部进入结果分析。①肌电图检测结果：肌萎缩侧索硬化症与脊髓型颈椎病患者的肌电图均呈神经源性损害改变，而肌萎缩侧索硬化症的损害更为广泛，尤其胸段棘旁肌自发电位的异常率高达93．3％（28/30）；脊髓型颈椎病患者的胸段棘旁肌自发电位异常率仅占3．3％（1／30）（P〈0．001）。肌萎缩侧索硬化症患者运动单位电位平均时限、波幅增高异常与脊髓型颈椎病比较有明显的差异，但在两病的鉴别诊断中并不具有特征性。②运动神经传导速度：肌萎缩侧索硬化症患者运动神经传导速度的复合肌肉动作电位的波幅下降率明显低于脊髓型颈椎病患者（75．6％，86．7％；X^2=7．25，P〈0．01）。运动传导速度减慢率也低于脊髓型颈椎病患者（14.4％，23．9％，X^2=5．18,P〈0．05）。⑧感觉神经传导速度：肌萎缩侧索硬化症与脊髓型颈椎病患者的感觉神经传导速度未受到影响。结论：①两组患者的肌电图均呈神经源性损害改变，而肌萎缩侧索硬化症患者胸段棘旁肌自发电位异常率明显高于脊髓型颈椎病。②神经电图显示两者感觉神经传导速度均未受到影响。⑨神经电图还显示肌萎缩侧索硬化症患者运动神经传导速度减慢和波幅下降的运动神经数均少于脊髓型颈椎病患者。     

电生理检查在格林-巴利综合征中的应用

目的探讨格林巴利综合征（GBS）肌电图及神经传导速度检测的特征及临床意义。方法对57例GBS患者的临床资料进行分析，并做针极肌电图（EMG）及神经传导速度（NCV）检测和分析，观察静息状态时自发电位，测定轻收缩时运动单位电位（MUP）的时限、波幅，大力收缩时的慕集相。测定运动、感觉神经的传导速度（MCV、SCV）及动作电位的末端潜伏期（ML）、波幅（AMP）、F波等。结果共检测164条肌肉、276条神经。EMG提示57例患者均呈神经原性改变；运动神经在下肢的异常率明显高于上肢，胫后神经异常率分别为：DM-lat 69．64％，AmP 66．07％。腓总神经异常率分别为：DM-lat 75．51％．MCV 55．10％，AmP 61．22％。所有被测运动与感觉神经的SCV、MCV异常率分别为54．79％与55．43％；10例（17．54％）F波未引出，38例（63．16％）F波潜伏期延长。结论GBS存在广泛的周围神经损害，肌电图和神经电图检查对GBS早期诊断和预后判断有重要指导价值。     

格林巴利综合征的临床与神经电生理分析

目的：总结格林巴利综合征（GBS）的临床和电生理参数异常分布情况,明确电生理对GBS的临床应用价值。方法：回顾分析38例GBS患者的临床及电生理资料,对运动神经传导速度（MCV）、F波、感觉神经传导速度（SCV）、肌电图的检测结果进行统计,观察其与临床表现的相关性。结果：本组患者感觉神经传导异常率低于运动神经传导异常率（P〈0．05）;上肢MCV异常率（24．40％）低于下肢（34．52％）（P〈0．05）;50％神经F波出波率降低或未引出波形;神经传导异常程度与脑脊液蛋白升高程度存在正相关关系;51．35％患者肌电图表现为神经源性损害;四肢远端肌肉正锐波、纤颤电位出现率（31．25％）与近端（21．57％）无统计学差异。结论：本组患者在电生理上既有神经脱髓鞘损害,亦有继发性轴索损害表现,远端运动神经损伤较感觉神经损伤重,下肢神经损伤较上肢重,神经损伤程度与病情相关,与病程未体现出明显的关系。     

糖尿病周围神经病变的电生理检测

目的:探讨电生理检测对糖尿病周围神经病的诊断意义。方法:对100例糖尿病患者测定正中神经肘→腕运动神经传导速度、波幅,指1→腕感觉神经传导速度、波幅。尺神经肘→腕运动神经传导速度、波幅,指5→腕感觉神经传导速度、波幅。胫后神经月国→踝运动神经传导速度、波幅,趾1→踝感觉神经传导速度、波幅。腓总神经膝→踝运动神经传导速度、波幅。腓肠神经外踝→腓肠点感觉神经传导速度及波幅。结果:糖尿病周围神经病以轴索及髓鞘同时受损多见,在四肢各神经中,感觉神经传导速度减慢及波幅降低较多见,复合肌肉动作电位波幅降低次之,而运动神经传导速度受累较少见。结论:神经电生理检查对糖尿病周围神经病诊断具有重要意义。     

2型糖尿病合并代谢综合征患者周围神经损害相关因素的研究

目的：运用神经传导检测,探讨合并代谢综合征（MS）是否加重2型糖尿病患者周围神经损害并分析相关因素。方法选取符合2型糖尿病诊断标准的患者258例,测定血压、体重、腰围、空腹血糖、胆固醇、甘油三酯、低密度脂蛋白、高密度脂蛋白、体重指数、神经传导,记录病史,根据 MS 诊断标准分为合并MS组145例和非合并MS组113例,并与100例健康人对照。结果与非合并MS组比较,合并 MS 组收缩压、体重、体重指数、腰围、感觉传导速度减慢、运动传导潜伏期延长及感觉运动传导波幅下降差异有统计学意义（P均＜0.05）;与对照组比较,合并MS组及非合并MS组均有感觉传导速度减慢、运动传导潜伏期延长及感觉运动传导波幅下降差异有统计学意义（P均＜0.05）。结论合并MS的2型糖尿病患者周围神经损害更重,可能与收缩压、体重、体重指数、腰围有关。     

Nerve conduction studies and vibration perception thresholds in diabetic and uremic neuropathy.

Two parameters of nerve conduction studies (nerve conduction velocities and amplitudes of the evoked sensory and motor responses) were compared with quantitative vibration perception thresholds (VPT) in patients with peripheral neuropathy (diabetes mellitus and/or end-stage renal disease). VPT measurements were made using a "two-alternative, forced-choice" method in which the patient is required to identify which of the two rods is vibrating at progressively decreasing vibration intensities. VPTs correlated significantly with nerve conduction velocities in all upper and lower extremity sensory and motor nerves tested, and with the amplitudes of the evoked motor responses in three motor nerves: median and ulnar (motor components) and tibial. For the median and ulnar nerves (motor components) the amplitudes of the evoked motor responses were more sensitive than nerve conduction velocities in correlation with VPTs. Comparison of VPT values, based upon whether or not evoked sensory and motor responses were obtained, indicated that mean VPTs were consistently higher among subjects in whom these evoked responses were not elicited. VPT measurements is thus shown to be a valid and valuable method for evaluation of severity in peripheral neuropathy. It has the advantages of being simple, quick and painless. Patient cooperation and compliance with this form of testing are excellent.     

Peripheral neuropathy associated with the hypereosinophilic syndrome

This case report documents the natural history of the peripheral polyneuropathy associated with persistent hypereosinophilia after treatment with corticosteroids. The neuropathy was documented by nerve conduction studies which revealed reduced amplitude of the sensory and motor evoked responses and slowed conduction velocities that were consistent with axon loss. Sural nerve biopsy revealed mild axonal loss distally without eosinophilic infiltration. The patient was treated with high-dose corticosteroids with a rapid normalization of his eosinophil count. Neurologic examination six months later revealed a slight improvement in his motor strength and reflexes but no change in sensory functions. Follow-up electromyographic evaluation demonstrated an average 89% improvement in evoked potential amplitudes and a slight improvement in distal latencies and conduction velocities. These findings suggest that the neuropathy associated with hypereosinophilia is axonal in nature and that there is a temporal relationship between reduction in the absolute eosinophil count and the improvement of the neuropathy.     

腓骨肌萎缩症1型临床与电生理分析

目的：分析腓骨肌萎缩症1型(charcot-marie-tooth disease，CMT1)患者的临床与电生理特点。方法：对1家系24人(已故2例)中7例(已故1例)CMT1患者临床特点进行总结，分析其中6例的电生理特征．包括肌电图(EMG)、运动神经传导速度(MCV)和感觉神经传导速度(SCV)。结果：4例于青少年期发病，有肢体远端肌无力和萎缩、腱反射减弱或消失、足畸形等典型的临床症状；3例EMG出现纤颤、正相电位，6例有运动电位时限延长；3例下肢SCV引不出，1例MCV引不出。结论：同一家系的CMT1型患者，临床表现亦差异较大；电生理特点为下肢神经病变重于上肢，感觉神经病变重于运动神经，且受累的严重程度不一致。     

Secondary changes in segmental neurons below a spinal cord lesion in man

The function of sensory and motor neurons below the level of a spinal cord injury (SCI) was assessed in 23 patients by recording the nerve conduction velocities and the amplitudes of evoked nerve and muscle compound action potentials for sural and posterior tibial nerves. Sensory and motor conduction velocities were generally normal when obtainable. Two patients had a unilateral reduction in the sural nerve and abductor hallucis muscle compound action potentials which may have been due to peripheral nerve compression. In 11 patients the abductor hallucis muscle compound action potential was below the normal range, while sural nerve action potentials remained within the normal range. Motor unit counts showed a reduction in the number of functioning motor units in these muscles.     

Herpes zoster of the head and limbs: electroneuromyographic and clinical findings in 158 consecutive cases

OBJECTIVES: To quantify electromyographic and neurographic changes and to correlate them with the clinical data of outpatients with herpes zoster. DESIGN: Prospective case series. SETTING: Outpatient department. PATIENTS: A consecutive, unselected series of 158 outpatient cases (88 women, 70 men; mean age, 64y) of herpes zoster of the head and limbs. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Blink reflex and electromyography and motor and sensory nerve conduction velocities of nerves and muscles corresponding to affected dermatomes. RESULTS: Postherpetic neuralgia (PHN), segmental zoster paresis, and polyneuropathy were found in 31%, 19%, and 2.5% of cases, respectively. Absence or reduction of sensory action potential amplitudes, blink reflex areas, and compound muscle action potential amplitudes were found in 60%, 31%, and 18% of cases, respectively. Sensory and motor conduction velocities and motor and blink reflex latencies were nearly always normal or only slightly slowed. Electromyographic signs of abnormal spontaneous activity were found in 36% of the cases. Electrophysiologic alterations were correlated among themselves, with age, with presence of segmental zoster paresis, and with absence of antiviral therapy. The extent of the skin rash (number of dermatomes affected by herpes zoster) was the only variable predictive of disappearance or improvement of PHN. CONCLUSIONS: Sensory axonal neuropathy, often associated with similar motor involvement, can be shown by classical electrophysiologic methods in herpes zoster. The severity of damage to motor fibers was related to damage to sensory fibers, but no relation was found between peripheral axon damage and PHN. The site of motor system damage may be the ventral roots, plexus, or peripheral nerve. The probability of complications and the severity of sensory and motor peripheral axonal damage were increased in older patients. Appropriate antiviral therapy seems to reduce the incidence of segmental zoster paresis and the severity of damage to the peripheral fibers. A reduced extent of herpetic rash was the only factor to correlate with a good outcome of PHN.     

持续性腹膜透析和血液透析对慢性肾功能衰竭多发性神经病转归的影响

目的通过电生理测试结果评估持续腹膜透析（PD）或血液透析（HD）的慢性肾功能衰竭患者周围神经病的发生率,并根据残余肾功能判断透析方式对周围神经功能的影响。方法将131例慢性肾功能衰竭患者按透析方式分为HD组（n=73）和PD组（n=58）。根据透析治疗期间肌电图的结果分别将HD组、PD组各分为MN亚组和非MN亚组。采用德国Medelec Shaphire 2MED仪对每例患者的正中神经、尺神经、腓总神经和胫神经的神经传导速度（NCV）进行电生理测定,感觉神经检测包括波幅、潜伏期和传导速度;运动神经检测则包括复合肌肉动作电位的波幅、潜伏期和传导速度。电生理检测发现至少2条神经异常结果可诊断为多发性神经病。在透析开始后的第1、6、12、18、24月检测每位患者的残余肾功能。结果 131例患者中在观察末期有78例存在感觉障碍。78例患者经肌电图检查诊断为MN者77例,其中HD组中MN患者44例（60.3%）,PD组中MN患者33例（56.9%）,2组比较差异无统计学意义（P〉0.05）。透析治疗初期HD组中的MN亚组、非MN亚组,PD组中的MN亚组、非MN亚组对残余肾功能（RRF）变化进行多重比较,差异均无统计学意义（均P〉0.05）。随着透析的进行,第12、24个月2种透析组的MN亚组RRF显著下降（P〈0.05）,非MN亚组RRF比较差异无统计学意义（P〉0.05）。结论 MN在慢性肾衰患者中是常见的并发症,发生率与治疗方法无明显关系;PD或HD治疗不能阻止慢性肾功能衰竭患者周围神经病变的发生。残留肾功能在保护慢性肾功能衰竭患者周围神经功能方面起了重要的作用。     

腕尺管综合征肌电图检测分析

目的探讨腕尺管综合征的肌电图表现。方法对13例腕尺管综合征患者行肌电图检测,包括尺神经运动潜伏期、运动波幅、尺神经小指感觉传导速度、感觉波幅等,与其健侧对照比较,评估肌电图表现差异。结果腕尺管综合征患者其患侧肌电图与健侧比较,尺神经运动潜伏期延长(P<0.01),运动波幅缩小(P<0.01),尺神经小指感觉传导速度减慢(P<0.01)、感觉波幅缩小(P<0.01),小指展肌、第Ⅰ骨间肌肌电图也出现静息状态及重收缩状态的改变。结论腕尺管综合征患者存在肌电图的多项改变,早期检测对于其诊断及其治疗具有重要意义。     

神经传导速度测定对糖尿病合并肘管综合征的诊断意义

目的对糖尿病并发肘管综合征患者的神经传导速度测定结果进行分析。方法对85例糖尿病患者行神经传导速度及肌电图检测,统计糖尿病周围神经损害及肘管综合征的比率。结果 85例患者中周围神经损害36例,符合肘管综合征诊断11例(12.9%),其中糖尿病周围神经损害合并肘管综合征7例,单纯肘管综合征4例,双侧均有损害的3例。肘管综合征表现为肘下-肘上运动神经传导速度减慢(同上臂比较>10m/s),并有腕—小指感觉神经电位波幅降低8例,肘下-肘上运动电位波幅降低(>50%)伴小指展肌、第一骨间肌出现自发电位3例。结论糖尿病患者中并发肘管综合征的患者并不少见,可能存在卡压机制,并且神经传导速度测定可以及早发现糖尿病并发的肘管综合征,使患者能及时得到治疗。     

Clinical and electrophysiological study of chronic inflammatory demyelinating polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP)is a set of chronic,immune－ mediated polyneuropathy.The incidence of this disorder is increasing in recent years, but so far its mechanism is not known well. In order to delineate its clinical and electrophysiological features,mechanism and treatment,11patients with CIDP were assessed clinically and electrophsiologically in present study. 2 Clinical data 2.1 General data We collected 52 patients with Guillain－ Barre Syndrome(GBS)f…