肌萎缩侧索硬化症患者的临床与神经电生理特点分析

目的：探讨肌萎缩侧索硬化症（ALs）患者的临床特点以及肌电图（EMG）和神经传导检测对ALS的诊断价值。方法：对56例ALS患者回顾性分析其临床特点、神经传导检测及其胸锁乳突肌、下胸段脊旁肌及肢体肌的EMG资料。结果：56例患者均呈广泛神经原性损害,胸锁乳突肌EMG异常率为89％,胸段脊旁肌EMG异常率为77％。两者异常率均低于上、下肢肌EMG的异常率（分别为100％,91％）。结论：早期EMG及神经传导检测有助于ALS的早期确诊,行胸锁乳突肌及下胸段脊旁肌EMG检测对ALs的诊断及鉴别诊断有重要价值。     

肌萎缩侧索硬化症患者临床与肌电图特点分析

目的：探讨肌萎缩侧索硬化症（ALS）患者的临床特点、肌电图（EMG）和神经传导速度（NCV）对ALS的诊断价值。方法：对42例ALS患者回顾性分析其临床特点、NCV及其胸锁乳突肌、胸段脊旁肌及肢体肌EMG资料。结果：42例患者均呈广泛神经原性损害,胸锁乳突肌EMG异常率为90％,胸椎旁肌EMG异常率为88％,两者异常率均低于上、下肢肌EMG的异常率（100％,98％）,而胸椎旁肌自发电位异常率又高于胸锁乳突肌。结论：早期EMG及NCV检查有助于ALS的早期确诊,行胸锁乳突肌及胸椎脊旁肌EMG检测对ALS的诊断及鉴别诊断有重要价值。     

神经电生理检测对肌萎缩侧索硬化症患者的早期诊断价值

目的：探讨肌萎缩侧索硬化症(ALS)与脊髓型颈椎病(CSM)的神经电生理改变的差异。方法：对60例ALS与CSM患者进行常规肌电图(EMG)、神经电图(ENoG)检测。EMG检测包括三肢体肌、胸锁乳突肌、胸段棘旁肌等肌肉，观察静息状态时自发电位，测定运动单位电位(MUP)的时限、波幅，大力收缩时的募集相。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)。结果：ALS与CSM中自发电位的异常率分别为：胸锁乳突肌39％和17．5％，胸段棘旁肌分别为93．3％和3．3％，肢体肌分别为88．1％和75％，ALS患者MUP时限、波幅的增高不同于CSM患者，差异均有显著意义。结论：胸段棘旁肌EMG阳性率高、简便易行，为早期鉴别诊断ALS与CSM提供了更敏感的依据。同时表明电生理检测可作为ALS可疑或ALS与CSM两病并存患者的随访观察及判定病情和预后的客观指标。     

肌萎缩侧索硬化患者的腹直肌肌电图检测

目的：探讨腹直肌针极肌电图（EMG）在肌萎缩侧索硬化（ALS）中的诊断价值。方法：对ALS组58例患者进行常规EMG检测,包括上、下肢肌肉、胸锁乳突肌、下胸段脊旁肌及腹直肌,测定自发电位、募集相、运动单位电位（MUP）时限、波幅及多相波,对健康对照组36例健康志愿者进行腹直肌EMG的检测,比较ALS组与对照组腹直肌EMG的差异,以及ALS组中腹直肌EMG与下胸段脊旁肌EMG的差异。结果：健康对照组腹直肌EMG的MUP参数分别为波幅（389±35．7）μV,时限（9．78±1．23）ms,多相波（16．2±5．2）％;ALS组腹直肌EMG的MUP参数分别为波幅（675±135）μV,时限（12．78±1．03）ms,多相波（36．2±7．2）％,ALS组腹直肌EMG各参数与健康对照组比较差异有统计学意义;ALS组腹直肌EMG神经原性改变（43／58,74％）与下胸段脊旁肌EMG（39／58,67％）比较差异无统计学意义。结论：腹直肌EMG各参数均能可靠、稳定地测出,可作为诊断ALS患者胸段下运动神经元病变的辅助手段。     

神经电生理检查在肌萎缩侧索硬化症中的应用

目的:观察神经电生理检查在肌萎缩侧索硬化症(amyotrophic lateral sclerosis,ALS)中的应用价值。方法:分别对28例临床确诊ALS、6例临床拟诊ALS、4例临床可能ALS患者进行4个区域的共8块肌肉肌电图(EMG)分析,四肢的磁运动诱发电位(MEP),上肢正中神经、尺神经、下肢胫神经F波检查,在双侧腓肠肌记录H波,四肢远端神经传导测定,包括运动传导速度(MCV)、感觉传导速度(SCV)、复合运动神经动作电位(CMAP)、感觉神经动作电位(SNAP)以及运动末梢潜伏期(DML)进行测定并分析,并与健康对照组30例进行比较。结果:临床确诊ALS的神经电生理测定各值异常均高于拟诊ALS和可能ALS(P<0.05),拟诊ALS和可能ALS组比较没有明显统计学差异。ALS组EMG异常率85%,MEP异常率72.4%,神经传导异常主要表现为CMAP降低36.2%,SCV基本正常,F波出波率下降33.3%,F波振幅增高26.3%,H波振幅增高26.3%。结论:EMG对ALS患者下运动神经元损害有定位诊断价值,EMG是ALS诊断的重要依据;MEP对ALS患者上运动神经元损害有诊断价值,但特异性不高;F波、H波对ALS患者上下神经元神经损害定位有补充诊断价值,神经传导测定用于ALS的鉴别诊断。     

运动神经元病的临床及神经电生理分析

目的:探讨本地区运动神经元病(MND)患者的临床表现及神经电生理特点。方法:对入选的21例MND患者进行肌电图(EMG)及神经传导检测(NCS),分析其临床特征及神经电生理特点。结果:在21例患者中,运动神经传导检测异常率为55.2%,主要表现为M波波幅降低;感觉神经传导正常率为98.85%。静息时发现纤颤电位18例(85.71%),正锐波16例(76.19%),束颤电位6例(28.57%),运动单位电位时限延长18例(85.71%),平均波幅增高10例(47.62%),大力收缩时表现为单纯相9例(42.86%)。其中上下肢肌肉的异常率为71.08%,胸锁乳突肌的异常率为92.31%,胸椎旁肌的异常率为33.33%。结论:神经电生理检查对MND的诊断非常重要,其中EMG检测是MND诊断的重要手段。EMG呈广泛性的运动神经元损害是MND的特征性改变,束颤电位在诊断中有重要价值。胸锁乳突肌阳性率高,可作为检查延髓节段下运动神经元受累的首选肌肉。     

52例肌萎缩侧索硬化症的临床及肌电图分析

目的：探讨肌萎缩侧索硬化（ALS）的临床表现及肌电图（EMG）特点,企为早期诊断提供依据。方法：回顾性研究近3年来收治的52例ALS患者的一般资料、临床表现、EMG及其它实验室检查,分析其发病特点、临床特征及EMG特点。结果：50例为散发病例,2例有ALS家族史。男性36例,女性16例,平均发病年龄54．67岁,平均病程13．69个月。起病形式均为缓慢起病,逐渐加重。几乎均以肢体肌无力、肌萎缩为首发症状,大部分患者肢体远端症状重于近端。主要临床表现为肢体肌和舌肌无力、萎缩及肌束震颤、锥体束征、构音障碍、呛咳及吞咽困难。无感觉障碍。EMG表现为广泛神经原性异常。颈椎磁共振（MRI）示74％的患者合并颈椎病。结论：ALS的诊断主要依靠临床表现及EMG检查。EMG、MRI及一些血液学检查是诊断及鉴别诊断该病的重要辅助检查手段。     

肌电图对肌萎缩侧索硬化症的早期诊断价值

目的:探讨肌萎缩侧索硬化症(ALS)患者的神经电生理特点及肌电图检测在肌萎缩侧索硬化症早期诊断中的临床意义。方法:对32例临床诊断为肌萎缩侧索硬化症的患者回顾性分析其神经传导速度检测和针极肌电图的检测。结果:32例ALS患者均呈广泛神经源性损害,其中胸锁乳突肌的异常率为93.8%,低于上下肢肌肉的异常率100.0%。结论:EMG和神经传导速度检测有助于ALS的早期诊断,而胸锁乳突肌的针极肌电图检测对ALS的诊断和鉴别诊断有重要的临床意义。     

肌电图

肌电图(electromyogram,EMG)是通过检测和研究肌肉生物电活动,借以判断神经肌肉系统机能变化的一门科学。广义EMG还包括重复电刺激及神经电图(神经传导速度、H反射、F波以及Blink反射等),肌电图检查结果要结合其他临床资料作综合分析。常用的EMG是以同芯针电极插入肌肉中,收集其附近肌纤维的电活动作分析,此外,还有单纤维EMG、巨EMG及扫描EMG。EMG检查的对象是运动单位电位(MUP),它是指一个下运动神经元所支配的肌纤维群所产生之综合电活动,每一个下运动神经元轴突所支配的肌纤维数目是不同的,可用神经支配值(IR)表示,正常肢体…     

肌萎缩侧索硬化症与脊髓型颈椎病的神经电生理鉴别诊断

目的：探讨肌萎缩侧索硬化症（ALS）与脊髓型颈椎病（CSM）的神经电生理鉴别诊断方法。方法：回顾性分析经临床确诊的23例ALS及28例CSM患者的神经电生理表现。结果：在神经电图检查中,ALS组除12例运动神经动作电位降低外,其余各项指标正常;CSM组病例中,既有运动及感觉神经传导速度减慢,也有动作电位降低及末梢潜伏期延长。肌电图（EMG）检查结果显示：ALS组异常率高于CSM组（P〈0．05）,且ALS组胸锁乳突肌及胫前肌异常率均显著高于CSM组（P〈0．01）,但胫前肌异常的4例CSM均合并有腰椎间盘突出。结论：对于临床拟诊为CSM患者有必要常规加作胸锁乳突肌EMG,这不仅有助于CSM与ALS的鉴别诊断,对于指导两者的治疗及预后判断也具有十分重要的意义。     

The pseudopolyneuritic form of amyotrophic lateral sclerosis (Patrikios' disease)

The line distinguishing motor neuron diseases (MNDs) from motor neuropathies is sometimes blurred. Among MNDs, the pseudopolyneuritic form of amyotrophic lateral sclerosis (ALS) strictly mimics a neuropathy. We describe the clinical and electrophysiological features in the early stages of the pseudopolyneuritic ALS, and assess the disease progression in eight patients. Early symptoms were unilateral foot-drop and, less commonly, paraparesis. At the clinical examination, weakness of distal and proximal leg muscles was often detected, while the hand muscles were rarely involved and craniobulbar muscles were spared. Definite upper motor neuron signs were rare in the early stages of the disease. Electromyography (EMG) showed active denervation in the lower limbs of all patients (distal > proximal) and in the paraspinal muscles of 7 patients (lumbosacral > thoracic), and more rarely in the upper limbs. Transcranial magnetic stimulation (TMS) yielded abnormal responses (low amplitude or absent cortical motor evoked potentials and prolonged central motor conduction time) in most lower-limb recordings, while mild abnormalities were rarely observed in the upper limbs. Haematologic and cerebrospinal fluid examinations were normal. Brain and spinal MRI showed no significant abnormalities. After a three years follow-up on seven patients, all cases were nonambulatory and had upper limb weakness, and most of them had bulbar dysfunction. The electrophysiological finding of both upper and lower motor neuron involvement of the lower limbs in the early stages of the disease could be a useful marker to distinguish the pseudopolyneuritic form of ALS from other MNDs and motor neuropathies.     

Histopathology of the late-onset motor neuron degeneration (Mnd) mutant in the mouse

The motor neuron degeneration (Mnd) is characterized by a progressive deterioration of motor function (stiff-legged gait, abnormal limb placements and grasping, and finally paralysis; moving from rear to forelimbs). There is a dramatic degeneration of spinal cord motor neurons, more severe in the lumbosacral than in the other regions, as well as variable pathology in the lower cranial nerves. Upper motor neurons of the red nucleus, reticular formation of the pons and medulla, and restricted areas of the cerebral cortex are also affected. Degenerating motor neurons share many characteristics seen in the human disease amyotropic lateral sclerosis, including loss of Nissl substance, increases in lipofuscin and abnormal cytoplasmic inclusions. Additionally, Mnd, like ALS, is a disease of later life.     

Shaping appropriate locomotive motor output through interlimb neural pathway within spinal cord in humans

Direct evidence supporting the contribution of upper limb motion on the generation of locomotive motor output in humans is still limited. Here, we aimed to examine the effect of upper limb motion on locomotor-like muscle activities in the lower limb in persons with spinal cord injury (SCI). By imposing passive locomotion-like leg movements, all cervical incomplete (n = 7) and thoracic complete SCI subjects (n = 5) exhibited locomotor-like muscle activity in their paralyzed soleus muscles. Upper limb movements in thoracic complete SCI subjects did not affect the electromyographic (EMG) pattern of the muscle activities. This is quite natural since neural connections in the spinal cord between regions controlling upper and lower limbs were completely lost in these subjects. On the other hand, in cervical incomplete SCI subjects, in whom such neural connections were at least partially preserved, the locomotor-like muscle activity was significantly affected by passively imposed upper limb movements. Specifically, the upper limb movements generally increased the soleus EMG activity during the backward swing phase, which corresponds to the stance phase in normal gait. Although some subjects showed a reduction of the EMG magnitude when arm motion was imposed, this was still consistent with locomotor-like motor output because the reduction of the EMG occurred during the forward swing phase corresponding to the swing phase. The present results indicate that the neural signal induced by the upper limb movements contributes not merely to enhance but also to shape the lower limb locomotive motor output, possibly through interlimb neural pathways. Such neural interaction between upper and lower limb motions could be an underlying neural mechanism of human bipedal locomotion.     

Histopathology of the late-onset motor neuron degeneration (Mnd) mutant in the mouse

The motor neuron degeneration (Mnd) is characterized by a progressive deterioration of motor function (stiff-legged gait, abnormal limb placements and grasping, and finally paralysis; moving from rear to forelimbs). There is a dramatic degeneration of spinal cord motor neurons, more severe in the lumbosacral than in the other regions, as well as variable pathology in the lower cranial nerves. Upper motor neurons of the red nucleus, reticular formation of the pons and medulla, and restricted areas of the cerebral cortex are also affected. Degenerating motor neurons share many characteristics seen in the human disease amyotropic lateral sclerosis, including loss of Nissl substance, increases in lipofuscin and abnormal cytoplasmic inclusions. Additionally, Mnd, like ALS, is a disease of later life.     

Histopathology of the late-onset motor neuron degeneration (Mnd) mutant in the mouse

The motor neuron degeneration (Mnd) is characterized by a progressive deterioration of motor function (stiff-legged gait, abnormal limb placements and grasping, and finally paralysis; moving from rear to forelimbs). There is a dramatic degeneration of spinal cord motor neurons, more severe in the lumbosacral than in the other regions, as well as variable pathology in the lower cranial nerves. Upper motor neurons of the red nucleus, reticular formation of the pons and medulla, and restricted areas of the cerebral cortex are also affected. Degenerating motor neurons share many characteristics seen in the human disease amyotropic lateral sclerosis, including loss of Nissl substance, increases in lipofuscin and abnormal cytoplasmic inclusions. Additionally, Mnd, like ALS, is a disease of later life.     

Time-course and characterization of orolingual motor deficits in B6SJL-Tg(SOD1-G93A)1Gur/J mice

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease affecting upper and lower motor neurons. Symptom onset may occur in the muscles of the limbs (spinal onset) or those of the head and neck (bulbar onset). Bulbar involvement is particularly important in ALS as it is associated with increased morbidity and mortality. The purpose of this study was to characterize bulbar motor deficits in the B6SJL-Tg(SOD1-G93A)1Gur/J (SOD1-G93A) mouse model of familial ALS. We measured orolingual motor function by placing thirsty mice in a customized operant chamber that allows for measurement of tongue force and lick rhythm as animals lick water from an isometric disc. Testing spanned the pre-symptomatic, symptomatic, and end-stage segments of the disease. Rotarod performance, fore- and hindlimb grip strength, and locomotor activity were also monitored regularly during this period. We found that spinal involvement was apparent first, with both fore- and hindlimb grip strength being affected in SOD1-G93A mice from the onset of testing (64 days of age). Rotarod performance was affected by 71 days of age. Locomotor activity was not affected, even near end-stage. Bulbar involvement appeared much later, with tongue motility being affected by 100 days of age. Tongue force was affected by 115 days of age. To our knowledge, these findings are the first to describe the onset of bulbar versus spinal motor signs and characterize orolingual motor deficits in this preclinical model of ALS.     

Increased creatine kinase and spontaneous activity on electromyography,in amyotrophic lateral sclerosis

INTRODUCTION: Mild to moderate elevation of muscle creatine kinase (CK) is commonly observed in amyotrophic lateral sclerosis (ALS). Although the determinants of increased the CK in ALS remain uncertain, we hypothesize that fasciculations and muscle denervation can be involved by damaging the muscle fibre. PATIENTS AND METHODS: We studied 87 ALS patients in whom CK determination was performed. In 47, a standardized EMG investigation was performed. In 22 patients a second CK determination was performed a mean of 5 months later. CK values were compared between different patients arranged in groups as determined by the number of regions with fasciculation as detected on the clinical examination, and the number of muscles with fasciculation or with fibrillation potentials as observed on EMG. RESULTS: 43% of our population had an increased CK value. Four out of 5 patients with suspected ALS had an increased CK value. The number of patients with increased CK value was not different between sexes, or between bulbar and spinal-onset patients. CK value was not related with disease duration, and did not change at the second measurement. CK value was not different between the groups studied. CONCLUSION: The fasciculations,and the signs of denervation on EMG, are not determinants for high CK values in ALS patients, which are still unknown. Increased CK can be useful in the differential diagnosis of patients with lower motor neuron disorders.