肌萎缩性侧索硬化症患者体感诱发电位研究

目的 研究肌萎缩性侧索硬化症(ALS)患者体感诱发电位(SEP)变化。方法 采用正中神经及肠后神经体感诱发电位(mSEP、tSEP)对30例患者进行检测，并与27例健康人作对比。结果 mSEP和tSEP的异常率分别为43．3％(13／30)及28％(7/5)，除N9、PF(腘点)、LP(T12点)峰潜伏期和对照组相比无显著差异外，其余各峰潜伏期及峰间期和对照组相比均有显著性差异。结论 ALS患者存在感觉通路损害，且中枢的改变较周围更明显，SEP检查对患者感觉损害的定位有一定价值。     

痛觉诱发电位示肌萎缩侧索硬化患者痛觉通路正常

目的 研究肌萎缩侧索硬化患者痛觉诱发电位的特点,评估其痛觉通路的传导.方法 肌萎缩侧索硬化患者60例,取卧位,应用接触性热痛诱发电位刺激器,直径27 mm(面积573 mm2),加热速度70℃/s.于54.5℃应用可调节脉冲,刺激部位为手背、前臂的掌侧面、第7颈椎棘突处(C7).记录仪器:Keypoint.net仪器.记录点为Cz和Pz.同时进行躯体感觉诱发电位检测,记录其波形及潜伏期.并对60名健康对照者进行相应研究.结果 肌萎缩侧索硬化患者接触性热痛诱发电位波形无异常,潜伏期分别为:手背刺激(561.2±28.6)ms,前臂掌侧刺激(540.1±39.2)ms,C7刺激(512.7±31.4)ms,与健康对照组[(558.7±30.2)、(536.6±23.5)、(501.8±26.0)ms]比较差异均无统计学意义(t=4.23、4.51、3.74,P＞0.05).其躯体感觉诱发电位各波潜伏期、波间期均正常.结论 肌萎缩侧索硬化患者接触性热痛诱发电位正常,提示其痛觉通路正常.     

肌萎缩侧索硬化与脊髓型颈椎病的鉴别诊断

目的　探讨肌萎缩侧索硬化 (ALS)与脊髓型颈椎病 (CSM )早期临床鉴别诊断方法。方法　回顾性对临床确诊的 36例ALS和 2 2例CSM主要症状 /体征、辅助检查异常率进行比较。结果　除肌无力为两病共同的常见症状和体征外 ,肌萎缩、肌束震颤、感觉异常、病理征阳性率皆有显著性差别 ;ALS不存在客观感觉障碍 ,CSM不存在构音 /吞咽障碍和掌颏反射阳性。运动单位减少 ,波幅增高 ,时限增宽 ,纤颤电位增多 ,感觉传导速度和体感诱发电位正常 ,胸锁乳突肌EMG异常均利于ALS诊断。结论　在临床早期 ,通过识别关键性的症状、体征 ,结合电生理等实验室检查 ,可以对ALS和CSM作出正确的鉴别诊断     

糖尿病患者中枢传导时间测定报告

本文报告８５例糖尿病患者中枢传导时间（ＣＣＴ）检测结果。作体感诱发电位刺激胫后神经时，周围神经部分和神经根的传导时间均明显延长，波幅下降。测定ＣＣＴ时Ｌ１－２椎旁横突处（ＦＯ）－Ｐ１，ＦＯ－Ｎ２的时间均延长，有显著差异。ＦＯ－Ｐ１波间期（ＩＰＬ）异常占４９％，ＦＯ－Ｎ２ＩＰＬ异常占９．４％，结果提示糖尿病时除周围神经受累外，中枢神经系统也有受累。     

脑血管病患者的经颅磁刺激运动诱发电位和短潜伏期体感诱发电位的对比研究

采用经颅磁刺激运动诱发电位（MEP）对72名脑血管病患者进行检测，其中57例还同时测定短潜伏期体感诱发电位（SSEP），并和50例正常人进行对比研究．结果发现脑血管病患者瘫痪侧上肢MEP异常表现为刺激无反应或皮层潜伏期延长和中枢传导时间延长，与正常对照组和健侧比较差异有极高度显著性（P＜0．001），瘫痪侧下肢MEP异常表现为刺激无反应或中枢传导时间延长，与正常对照组和健侧比较，差异有显著性（P＜0．05）．MEP和SSEP异常率在脑出血与脑梗塞之间无显著性差异（P＞0．05），而与临床表现和病变部位密切相关。本文提示MEP和SSEP分别能客观地反映脑血管病愈者中枢运动传导通路和中枢感觉通路功能受损的情况．     

神经传导速度在肌萎缩侧索硬化中的诊断价值

目的　研究肌萎缩侧索硬化 (ALS)患者中神经传导速度的改变 ,建立量化评定肌萎缩侧索硬化病情轻重的神经生理指数。方法　对 2 1名ALS患者的 3 0条尺神经、3 2条正中神经及 2 4名健康对照组的 3 8条尺神经、40条正中神经进行神经运动传导速度 (MCV)及感觉传导速度 (SCV)和F波的检测。两组间数据进行统计学分析。结果　ALS组正中神经、尺神经运动传导速度的远端潜伏期 (DML)、肌肉动作电位 (CAMP波幅及面积、F波的出现率 )较对照组有显著性差异。而两组MCV、SCV、F波的潜伏期差异无显著性。ALS组中 1 0名小指展肌的肌力与 (CAMP波幅 /DML×F出现率 )的数值有显著的相关性 (r=0 89,P <0 0 1 )。结论　 (CMAP波幅 /DML×F波的出现率 )是一种有效的客观的电生理指数 ,可对ALS病情进行量化评估     

肌萎缩侧索硬化症与脊髓型颈椎病临床和肌电图特点相关性研究

目的肌萎缩侧索硬化症与颈椎病脊髓型患者的临床特点、肌电图和躯体感觉诱发电位的相关性研究。方法对38例肌萎缩侧索硬化症和脊髓型颈椎病患者的临床特点进行分析,并在肌电图及躯体感觉诱发电位、影像学检查进行比较。结果肌萎缩侧索硬化症患者肌电图呈广泛神经源性损害;脊髓型颈椎病患者受损害的神经根呈节段性分布,躯体感觉诱发电位有助于脊髓型颈椎病的诊断,影像学检查可见颈髓受压。结论肌电图及躯体感觉诱发电位、影像学检查是几种敏感的检测手段,在肌萎缩侧索硬化症与脊髓型颈椎病的诊断和鉴别诊断中有重要意义。     

肌萎缩侧索硬化症的诱发电位改变

目的　探讨肌萎缩侧索硬化症 (ALS)的诱发电位改变。方法　对 31例ALS患者中 2 1例进行体感诱发电位 (SEP)、2 8例进行听觉诱发电位 (BAEP)、30例进行视觉诱发电位 (VEP)检查 ,并分别与年龄、身高相匹配的健康者进行比较。结果　 31例患者中 3例存在传导束型感觉障碍 ;SEP示N13 ～N2 0 IPL为 (6 9± 1 4 )ms ,较对照组显著延长 (P <0 0 1) ;2 8例BAEP均正常 ;30例VEP示P10 0 IPL为 (10 2 1± 5 8)ms,较对照组显著延长 (P <0 0 1)。结论　SEP检查可为ALS涉及感觉损害提供客观依据     

躯体感觉诱发电位在糖尿病性脊髓病中的应用

目的探讨躯体感觉诱发电位(SEP)与糖尿病合并深感觉障碍的关系,揭示SEP对糖尿病合并深感觉障碍的定位价值,并将诱发电位结果与神经传导(NCV)测定结果进行比较,分析二者在糖尿病性神经系统病变中的关系。方法对52例糖尿病患者及40例正常人进行双侧胫神经躯体感觉诱发电位测定,对两组SEP各波潜伏期及波幅的均值进行t检验。同时对52例患者均进行双下肢周围神经传导速度测定,结果与SEP进行对比分析。结果 2组SEP的P40潜伏期及波幅比较差异有统计学意义(P<0.01),N9波幅及潜伏期比较差异无统计学意义(P>0.05),SEP与NCV不相关(P>0.05)。结论 SEP为糖尿病并发深感觉障碍中枢段病变提供了早期诊断的客观依据,SEP与NCV检测不相交。     

平山病的神经电生理学研究

目的 探讨平山病的神经电生理学特点及其与肌萎缩侧索硬化、多灶性运动神经病的鉴别诊断.方法 分别对平山病(26例)、肌萎缩侧索硬化(30例)和多灶性运动神经病(16例)患者进行运动和感觉传导速度、肌电图及交感皮肤反应等神经电生理学检查.运动传导速度采用由远端至近端分段刺激,记录复合肌肉动作电位的波幅、时限、面积及波形的变化,并判断是否存在神经传导阻滞;肌电图检查记录脑区肌肉(双侧胸锁乳突肌),颈区肌肉(拇短展肌、小指展肌、第一骨问肌、肱二头肌),胸区肌肉(T10椎旁肌、腹直肌)和腰骶区肌肉(胫骨前肌)的肌电活动.比较3组患者神经电生理学特点的差异性.结果 平山病组患者均无神经传导阻滞,肌电图检查显示颈区肌肉呈神经源性损害;肌萎缩侧索硬化组患者亦无神经传导阻滞,肌电图检查显示脑区、颈区、胸区和腰骶区肌肉均呈神经源性损害;多灶性运动神经病组患者均存在神经传导阻滞,肌电图检查颈区和腰骶区肌肉呈神经源性损害.平山病组患者神经传导阻滞的发生率与多灶性运动神经病组比较,差异有统计学意义(x2=42.000,P=0.000);平山病组患者神经源性损害的发生率与肌萎缩侧索硬化组比较,差异亦有统计学意义(x2=56.000,P=0.000).结论 平山病组患者运动和感觉传导速度均无异常,无神经传导阻滞,但肌电图检查显示颈区肌肉呈神经源性损害.     

Abnormalities of multimodality evoked potentials in amyotrophic lateral sclerosis

Thirty-two patients with amyotrophic lateral sclerosis were studied with somatosensory evoked potentials (SEPs), visual evoked potentials, and brain-stem auditory evoked potentials. H-reflexes were used to screen for abnormalities of peripheral nerve conduction. Nineteen patients (59%) showed an abnormality of lower extremity SEPs. In 13 patients (40%) the delay was of central origin, while in six patients (19%) peripheral conduction delay was possible. Abnormality of upper limb SEPs was seen in 11 patients (34%), all but two of whom had abnormal lower limb SEPs as well. Four patients (12%) had abnormal brain-stem auditory evoked potentials, all of whom had abnormal SEPs from upper and lower limbs. Four patients had abnormal visual evoked potentials, which in three patients were of minor degree. These results give physiologic evidence to suggest that abnormalities in amyotrophic lateral sclerosis occur outside the motor system.     

Somatosensory evoked potentials in syringomyelia.

The two types of upper limb somatosensory evoked potential abnormality observed in nine patients with syringomyelia were reduced amplitude or absent cervical potentials and an abnormal central conduction time. Although this pattern of abnormalities resembles that observed in other intrinsic spinal cord lesions, it differs from peripheral nerve diseases and cervical radiculopathy in which the central conduction time is normal.     

The diagnostic value of sensory evoked potentials in pediatric Wilson disease

We studied the sensory evoked potentials in pediatric Wilson disease to verify their subclinical neurologic involvement and to elucidate the role of cirrhosis in abnormal evoked potentials in non-neurologic Wilson disease. Thirty children (17 male, 13 female), diagnosed with Wilson disease before 18 years, were enrolled. The mean age during studies was 15.8 +/- 6.3 years, and disease duration since diagnosis was 3.0 +/- 3.3 years. In 12 neurologic Wilson disease cases, there were prolonged interpeak latencies of brainstem auditory evoked potentials III-V, I-V, somatosensory evoked potentials N13-N20 (P < 0.01 vs controls and non-neurologic cases), and P100 latency (P < 0.01 vs controls). All 12 patients had at least one abnormal evoked potential, including 91.7% brainstem auditory, 58.3% somatosensory, and 25% visual evoked potentials. In 18 non-neurologic Wilson disease cases, there were still prolonged interpeak latencies for brainstem auditory evoked potentials I-V and somatosensory evoked potentials N13-N20 (P < 0.05 vs controls), with 27.8% of them having at least one abnormal evoked potential, including 16.6% brainstem auditory, 5.6% somatosensory, and 11.1% visual evoked potentials. In those with non-neurologic Wilson disease, there were no significant differences in all the evoked potential parameters between the cirrhotic and non-cirrhotic patients.     

Cervical cord dysfunction during neck flexion in Hirayama's disease

Neck flexion may play a role in the pathogenesis of Hirayama disease. Upper limb somatosensory evoked potentials were recorded in five patients with Hirayama disease, six patients with ALS, and 14 healthy subjects. Neck flexion caused a significant amplitude decrease of the N13 cervical response only in patients with Hirayama disease. Direct cord compression or microvascular changes can in theory account for this position-related dysfunction.     

Motor and somatosensory evoked potentials in tuberculous meningitis: a clinico-radiological correlation

OBJECTIVE: The sensory and motor functions in severe tuberculous meningitis (TBM) may be difficult to assess clinically and may be helped by evoked potential studies. Lack of motor and somatosensory evoked potential studies in TBM prompted the present study. METHODS: All the patients with TBM underwent detailed neurological evaluation and cranial CT scan study. Motor and somatosensory evoked potentials to both upper and lower limbs were carried out bilaterally. The outcome was defined on the basis of 3 month Barthel Index (BI) score into good (BI > 12) and poor (BI < 12). RESULTS: Forty-one highly probable patients with TBM whose ages ranged between 8 and 64 years and 14 of whom were females were included in this study. Twenty-three patients were in stage III (meningitis, neurological signs and altered sensorium), 12 in stage II (meningitis with neurological sign) and the remaining patients were in stage I (meningitis only). Cranial CT scan was carried out in all and MRI in 18 patients. On CT scan hydrocephalus was present in 21, infarction in 14 and tuberculoma in 4 patients. Motor evoked potential (MEP) was abnormal in 18 patients (36 limbs) and SEP in 9 patients (23 limbs). Upper limb central motor conduction time to abductor digiti minimi (CMCT-ADM) was abnormal in 15 and that to tibialis anterior (TA) in 21 limbs. CMCT abnormality was lateralized in 6 and only upper or lower limbs were involved in 11 patients. The SEP abnormalities were lateralized in 2 patients and only upper or lower limbs were involved in 3. The MEP changes correlated with stage of TBM and outcome whereas SEP with outcome only. CONCLUSION: Motor and somatosensory evoked potentials may be helpful in objective documentation of respective motor and sensory functions in TBM patients with altered sensorium.     

Adrenoleukodystrophy and variants. Clinical, neurophysiological and biochemical studies in patients and family members

Clinical, neurophysiological and biochemical studies were performed in patients with various forms of adrenoleukodystrophy (ALD) and their family members. The patients showed an abnormality in saturated very long chain fatty acids and in the somatosensory and brain stem auditory or visual evoked potentials. Female presumptive carriers without abnormal neurological manifestations also showed abnormality in the somatosensory or brain stem auditory evoked potentials and in saturated very long chain fatty acids. One ALD patient and his mother, a female carrier, had the decreased beta-galactosidase activity. The increase in saturated very long chain fatty acids was found, not only in sphingomyelin, but also in phosphatidylcholine and phosphatidylserine. Our results suggest that a generalized abnormal metabolism of VLFA and an abnormality in the central nervous system exist in our patients and female carriers.     

Somatosensory evoked response abnormalities in high-risk newborns

In a previous study from our laboratory, the prognostic significance of the auditory brainstem evoked response was assessed in high-risk neonates. An abnormal auditory brainstem evoked response predicted neurologic deficits at age 1 year; however, a normal result did not predict a normal outcome. In order to evaluate the prognostic utility of examining other sensory pathways, somatosensory evoked responses were elicited following median nerve stimulation. Testing was performed at 37–44 weeks conceptional age (defined as gestational age plus chronologic age) and at 2 and 6 months conceptional ages. Those patients studied included 34 high-risk neonates and 18 healthy, term infants as controls. Ten of the 34 patients had abnormal somatosensory evoked responses. Abnormalities included increased absolute (N19, P22) and interwave (N13–N19, N19–P22) latencies and flat potentials, alone or in combination. Three children with flat potentials demonstrated a persistence of this abnormality on subsequent examination and they later presented clinically with spastic quadriparesis. Four infants with increased latencies manifested normal responses on subsequent examination. Recently, these 4 patients exhibited tone abnormalities and mild developmental deficits; developmental outcome, however, will be assessed in a blind study at 1 year of age as part of this ongoing prospective study. Preliminary results suggest that somatosensory evoked responses may be valuable as an electrophysiologic predictor of outcome.     

Electrophysiologic characterization in spinocerebellar ataxia 17

The authors performed a multimodal electrophysiologic evaluation in nine patients belonging to four SCA17 (spinocerebellar ataxia type 17) families. Peripheral nerve and visual system were not involved. Brainstem auditory evoked potentials were constantly abnormal with central type lesions. Magnetic motor evoked potentials were abnormal only in the lower limbs, suggesting a length-dependent involvement of the pyramidal tract. Somatosensory evoked potentials were abnormal in almost all our patients, and abnormalities were consistent with a somatosensory pathway involvement along the brainstem.