The diagnostic reliability of magnetically evoked motor potentials in multiple sclerosis.

In a prospective study, we evaluated the technique of magnetically evoked motor potentials (MEP) in the diagnosis of multiple sclerosis (MS). We consecutively included 68 patients with symptoms or signs compatible with a demyelinative CNS affection. We subjected all patients to CSF analysis, MRI studies of the brain and brainstem, visual evoked potentials (VEP), brainstem auditory evoked potentials (BAEP), and somatosensory evoked potentials (SSEP). We then used the results to categorize the patients according to the Poser criteria of multiple sclerosis. Blinded from the results of the above investigations, one of the authors made MEP recordings from three muscles in the upper limbs and two in the lower limbs in all 68 patients. Forty patients received an MS diagnosis, and in these, MRI was positive in 88%, MEP in 83%, VEP in 67%, SSEP in 63%, and BAEP in 42%. As to the diagnosis of MS, the reliability of a prolonged central motor conduction time (CMCT) was 0.83 (0.73 to 0.93), while the reliability of a normal CMCT was 0.75 (0.61 to 0.98). The information gained by MRI was best supplemented by VEP. Of the neurophysiologic tests, the MEP was in closest agreement with the MRI with a concordance of 85%.     

Evoked potential studies in Friedreich's ataxia and progressive early onset cerebellar ataxia

We recorded somatosensory evoked potentials (SEP) in 15 patients affected by Friedreich's ataxia (FA) and in 9 patients with progressive early onset cerebellar ataxia (PEOCA). Brainstem auditory evoked potentials (BAEP) were also recorded in 14 FA patients and in five PEOCA patients. SEP results showed clear differences between groups of FA, evidence of peripheral involvement was seen in all patients, with absence of the N9 potential or a major reduction of its amplitude. In patients in whom central responses could be recorded, conduction velocity was normal or near normal up to the brainstem but was reduced from brainstem to cerebral cortex. Four patients with PEOCA had SEP abnormalities similar to those seen in FA. In the five other patients, the amplitude and latency of N9 were normal but conduction velocity was reduced from brainstem to cerebral cortex. In FA, BAEP were abnormal in all patients with a disease duration of four years or more but were normal in four of the five PEOCA patients. Systematic evoked potential recording is useful in the investigation of hereditary ataxias.     

Evaluation of central neuropathy in type II diabetes mellitus by multimodal evoked potentials

The electrophysiological results in 51 patients with diabetes mellitus type II were compared with those in 30 age and sex matched healthy control subjects. Peripheral and cortical latencies of median and tibial somatosensory evoked potentials (SEP), bilateral I-III and I-V interpeak latencies (IPL) of brainstem auditory evoked potentials (BAEP), bilateral P100 latency of visual evoked potentials (VEP) and bilateral cortical latency and central motor conduction time of motor evoked potentials (MEP) were evaluated. We observed prolonged latencies suggestive of central neuropathy in DM type II. It has been shown that most of the electrophysiological parameters in patients with DM type II correlate with the duration of the disease, some of them with the age of the patient, and few of them with the onset of the disease. To our knowledge, there is no correlation between the electrophysiological parameters and the level of glycemia or the degree of metabolic control. We conclude that central and peripheral neuropathies in DM are related to the duration of the disease and not to the degree of hyperglycemia and metabolic control.     

脊髓型颈椎病病人经颅磁电刺激运动诱发电位的对比研究

目的探讨磁电刺激运动诱发电位（ＭＥＰ）在脊髓型颈椎病（ＣＳＭ）的应用价值，并对其临床相关性进行分析。方法采用经颅磁、电刺激对３０例脊髓型颈椎病病人以及年龄性别等相配匹的３０名健康成人分别于外展小指肌、肱二头肌及下肢展短肌表面进行ＭＥＰ的检测。结果全部病人的ＭＥＰ都出现异常，表现为潜伏期、中枢传导时间（ＣＭＣＴ）延长，时限增宽，波辐降低或不能引出。磁刺激ＭＥＰ的ＣＭＣＴ和皮层刺激潜伏期与脊髓型颈椎病临床日本整形外科协会（ＪＯＡ）评分间有密切相关性，能较好地反映ＣＳＭ病人的病情。结论ＭＥＰ在检测ＣＳＭ病人运动功能方面具有定量评价作用。与电刺激相比，磁刺激ＭＥＰ能更好地反映ＣＳＭ病人的病情。     

Correlation between disability and transcranial magnetic stimulation abnormalities in patients with multiple sclerosis

Multiple sclerosis (MS) is an idiopathic inflammatory demyelinating disorder of the central nervous system. Clinical evaluation, MRI, cerebrospinal fluid testing and evoked potentials (EP) are among the available methods utilized for disease diagnosis and monitoring. To date, no surrogate markers have been established to assess disease evolution and progression. The aim of this study is to assess motor evoked potentials (MEP) of MS patients by transcranial magnetic stimulation (TMS) and investigate the possible correlations between TMS abnormalities and disability in the patient group, which includes a subgroup with no apparent pyramidal tract dysfunction. A total of 131 clinically definite MS patients were included in the study. Motor responses to TMS stimulation were recorded. Absent values, decreases in amplitude, prolongation of latency and central motor conduction time (CMCT) were considered as abnormal. A total of 109 (83%) patients displayed abnormal MEP amplitude, 68 (52%) displayed MEP latency, and 64 (49%) displayed CMCT abnormalities. Abnormal CMCT, latency and amplitude results were correlated with Expanded Disability Status Scale scores (p < 0.001). Our results indicate that TMS-EP in MS patients is correlated with disability, and that these findings may support the role of EPs in predicting disability even in subclinical presentations.     

Transcranial magnetic stimulation in Beh?et's disease: a cross-sectional and longitudinal study with 44 patients comparing clinical, neuroradiological, somatosensory and brain-stem auditory evoked potential findings.

OBJECTIVES: To compare neurological involvement in Beh?et's disease as documented by transcranial magnetic stimulation (TMS) with clinical, neuroradiological, somatosensory (SEP) and auditory evoked potential (BAEP) findings. METHODS: Forty-four patients were studied over an 8 year period. Nine patients had follow-up studies done. TMS central motor conduction (CMC) studies to upper and lower limb muscles, brain magnetic resonance imaging (MRI), SEP, and BAEP testing were conducted. RESULTS: Thirty-nine patients had CMC slowing, decreased amplitude or absent motor evoked potentials (MEP); 5 of these patients were neurologically normal. Concordance of TMS results, clinical deficits, and MRI findings occurred in 36 of the 39 patients. SEP and BAEP testing proved non-complementary to MEP. Generally, follow-up studies revealed faster CMC and higher MEP amplitude. However, in two patients the CMC time to one target muscle became prolonged with diminished MEP amplitude over a period of 1.5-3 years. CONCLUSIONS: TMS can be useful in detecting and quantifying motor tract dysfunction in Beh?et's disease and provides functional information complementary to imaging studies. TMS is more sensitive than either SEP or BAEP. Our longitudinal studies suggest that TMS studies may be valuable in monitoring disease activity or therapeutic response.     

支架置入术对椎基底动脉系统短暂性脑缺血发作患者诱发电位的影响

目的 观察支架置入术能否改善椎基底动脉系统短暂性脑缺血发作（TIA）患者的亚临床症状。方法 11例症状性椎基底动脉狭窄的椎基底动脉系统TIA患者,支架置入术前后分别检测体感诱发电位（SEP）、脑干听觉诱发电位（BAEP）、视觉诱发电位（VEP）,记录各诱发电位的潜伏期及波幅。结果 （1）术前诱发电位均异常,主要表现为SEP N20及P40潜伏期异常,BAEPⅠ、Ⅲ、Ⅴ波潜伏期延长,VEP P100潜伏期延长。（2）与术前相比,术后1周BAEP表现为Ⅰ～Ⅲ波潜伏期缩短（P =0.046）、Ⅲ波幅升高（P =0.05）;SEP表现为N20潜伏期缩短（P =0.012）,N13～N20间期缩短（P =0.013）,P14～N20间期缩短（P =0.005）;VEP表现为 P100潜伏期缩短（P =0.022）。结论 支架置入术后,椎基底动脉系统TIA患者的SEP、BAEP、VEP好转,提示患者的亚临床症状恢复。     

Mechanism of prolonged central motor conduction time in compressive cervical myelopathy.

OBJECTIVE: To investigate the mechanism of prolonged central motor conduction time (CMCT) in compressive cervical myelopathy, we compared the calculated CMCT following transcranial magnetic stimulation (TCM) and evoked spinal cord potentials (ESCPs) following transcranial electric stimulation (TCE). METHOD: Motor evoked potentials following TCM were recorded from abductor digiti minimi and abductor hallucis brevis muscles in 16 patients with compressive cervical myelopathy. CMCT was calculated by subtracting peripheral conduction time using peripheral nerve stimulation from MEP latency. ESCPs following TCE were recorded intraoperatively from posterior epidural space. RESULTS: CMCT was prolonged and significant attenuation of the ESCP amplitude following TCE was observed in all patients with cervical myelopathy. In 8 of 16 patients CMCT was significantly prolonged but ESCPs were recorded at the C6-7 level with normal negative peak latency. CONCLUSIONS: Prolonged CMCT may occur with only a minor amount of conduction slowing in the corticospinal tract in compressive cervical myelopathy. Impaired temporal summation of multiple descending potentials following TCM produced delays of motor neuron firing that contribute to the mechanism of prolonged CMCT.     

Prolonged central motor conduction time of lower limb muscle in spinocerebellar ataxia 6.

We investigated the function of corticospinal tract in spinocerebellar ataxia 6 (SCA6) by measuring the central motor conduction time (CMCT). Motor evoked potentials (MEP) of tibialis anterior (TA) muscle were elicited by magnetic stimulation to motor cortex and spinal cord in 9 SCA6 patients and 10 normal height- and age-matched subjects. CMCT in lower limb of SCA6 patients (18.1+/-1.9 ms) was significantly prolonged than that of the normal subjects (15.0+/-1.0 ms) ((p < 0.001). The prolonged CMCT was well correlated with the duration of disease (p = 0.005), but MEP amplitudes and stimulation intensities were not significantly different. These results indicate that the corticospinal tract function is also impaired and correlate with the disease duration in SCA6.     

Central motor conduction to upper and lower limbs after magnetic stimulation of the brain and peripheral nerve abnormalities in 20 patients with Friedreich''s ataxia

Central motor conduction time (CMCT) to thenar and soleus muscles was measured after magnetic stimulation of the cortex in 20 cases of Friedreich's ataxia (FA) and was abnormal in all. CMCT values were related to disease duration and disability. The amplitude of CMAP after cortex stimulation was severely reduced in the most disabled patients. Reduction in amplitude of the nerve evoked potentials was related neither to disease duration nor grade of disability. These results suggest that clinical worsening in FA is mainly due to progressive central motor pathway involvement. CMCT study is a better index of disease progression than peripheral nerve examination. Abnormalities in CMCT may be the third electrophysiological diagnostic criterion in FA, after reduced amplitude of nerve action potentials and absent H reflex.     

Characteristic evoked potentials in childhood-onset dentatorubral-pallidoluysian atrophy

To determine the characteristics of multimodal evoked potentials (MEPs) in childhood-onset dentatorubral-pallidoluysian atrophy (DRPLA) we studied three DRPLA patients with progressive myoclonus epilepsy. Brainstem auditory evoked potentials showed reduced or absent brainstem components as well as delayed latencies. In addition, short latency somatosensory evoked potentials (S-SEPs) had prolonged central conduction time and reduced amplitude of cortical components. Two patients with symptom onset in the first decade of life had extremely enlarged flash visual evoked potentials with shortened latency even in the absence of giant SEPs. Therefore, children with progressive myoclonus epilepsy and the above MEP findings are likely candidates for childhood-onset DRPLA and should undergo DNA analysis for DRPLA.     

Changes of brainstem auditory and somatosensory evoked

Objective: to investigate the characteristics and clinical value of evoked potentials in late infantile form of metachromatic leukodystrophy. Methods: Brainstem auditory, and somatosensory evoked potentials were recorded in 6 patients, and compared with the results of CT scan. Results: All of the 6 patients had abnormal results of BAEP and MNSEP. The main abnormal parameters in BAEP were latency prolongation in wave I, inter-peak latency prolongation in Ⅰ-Ⅲ and Ⅰ-Ⅴ. The abnormal features of MNSEP were low amplitude and absence of wave N9, inter-Peak latency prolongation in Ng-N13 and N13-N20, but no significant change of N20 amplitude. The results also revealed that abnormal changes in BAEP and MNSEP were earlier than that in CT. Conclusion: The detection of BAEP and MNSEP in late infantile form of metachromatic leukodystrophy might early reveal the abnormality of conductive function in nervous system and might be a useful method in diagnosis.     

Transcranial magnetic stimulation: role in the evaluation of disability in multiple sclerosis

BACKGROUND: In patients with multiple sclerosis (MS), transcranial magnetic stimulation (TMS) has shown significant prolongation of central motor conduction time (CMCT). Abnormal CMCT may reflect sub-clinical involvement of motor pathways and correlate with clinical motor disability. OBJECTIVE: To determine the diagnostic yield of TMS in MS and the possible correlation of TMS abnormalities with clinical disability. MATERIALS AND METHODS: Thirty patients with clinically definite MS presenting in acute relapse or with progressive disease course and 30 healthy controls were evaluated. TMS parameters evaluated included threshold intensity, motor evoked potentials (MEP) amplitudes and latencies and CMCT. Reassessment studies were done after three months. STATISTICAL ANALYSIS: Student t-test, Mann-Whitney U test and Spearman's rank correlation test were used to assess the relationships. RESULTS: Patients with MS had significantly higher threshold intensities, prolonged CMCT and reduced MEP amplitudes as compared to controls. Abnormalities in at least one parameter were observed in 86.7% of patients. When inter-side asymmetries in MEP latency and/or in CMCT were considered, the diagnostic yield increased to 96.7%. The diagnostic yield was 74.7% for visual evoked potentials, 13.3% for brainstem auditory evoked response and 10% for cerebrospinal fluid oligoclonal band. One MS patient without pyramidal or cerebellar dysfunction had prolonged CMCT. CMCT abnormalities correlated significantly with the degree of pyramidal signs, limb ataxia, intention tremor, dysdiadokokinesia and overall cerebellar score. In patients who had clinical improvement, follow-up studies showed improvement in CMCT parameters. CONCLUSION: TMS is a highly sensitive technique to evaluate cortico-spinal conduction abnormalities in MS that may have no clinical correlate and in monitoring the course of the disease. The effects of cerebellar dysfunction on TMS results need further evaluation.     

三重磁刺激技术对上运动神经元损伤的评价作用

目的研究三重磁刺激技术(TST)的特点,探讨TST在评价运动神经元疾病患者上运动神经元(UMN)损害中的作用。方法选取肌萎缩侧索硬化(ALS)患者12例、下运动神经元综合征(LMNS)患者14例,分别进行双上肢小指展肌TST测定及运动诱发电位(MEP)潜伏期、中枢运动传导时间(CMCT)测定,并将TST波幅比与改良的医学研究委员会(MRC)评分、修订的肌萎缩侧索硬化功能评分量表(ALSFRS-R)评分、肺功能百分比、病程等进行相关性分析。结果 ALS患者12例均完成双侧TST检测,其尺神经TST波幅比为(55.8±32.6)%,较正常参考值下降(F=-3.448,P=0.002),TST波幅比阳性率为70.8%。LMNS患者共13例完成TST检测,其尺神经TST波幅比为(79.4±16.4)%,与正常参考值无统计学差异(F=0.215,P=0.832)。ALS组尺神经TST波幅比〔(55.8±32.6)%〕低于LMNS〔(79.4±16.4)%〕(F=-3.275,P=0.002)。TST波幅比评价UMN体征的敏感度76.5%,高于传统电生理指标;特异度为54.5%,低于传统电生理指标。TST波幅比与改良的MRC评分存在相关性(r=0.431,P=0.035),与ALSFRS-R评分、肺功能、病程、MEP潜伏期以及CMCT均无相关性。结论 TST可以发现亚临床UMN损害,其敏感性高于传统电生理观测指标。TST可以定量评估UMN受损程度,有可能用于ALS患者病情监测。     

Efferent and afferent evoked potentials in patients with adrenomyeloneuropathy

Objective

This paper investigates efferent and afferent conductions of the central nervous system by various evoked potentials in patients with adrenomyeloneuropathy (AMN).

Patients and methods

Ten pure AMN patients without cerebral involvement were studied. Motor evoked potentials (MEPs), somatosensory evoked potentials (SEPs), auditory brainstem response (ABR), and pattern reversal full-field visual evoked potentials (VEPs) were recorded. For MEP recording, single-pulse or double-pulse magnetic brainstem stimulation (BST) was also performed.

Results

Abnormal MEP was observed in all ten patients, abnormal SEP in all ten, abnormal ABR in nine, and abnormal VEP in only one. Brainstem latency was measured in three of the seven patients with central motor conduction time (CMCT) prolongation. The cortical–brainstem conduction time was severely prolonged along the normal or mildly delayed brainstem–cervical conduction time in those three patients.

Conclusions

The pattern of normal VEP and abnormal MEP, SEP, ABR is a clinically useful electrophysiological feature for the diagnosis. BST techniques are helpful to detect, functionally, intracranial corticospinal tract involvement, probably demyelination, in pure AMN patients.     

Magnetic stimulation study in patients with myotonic dystrophy

To further define motor nervous system alterations in myotonic dystrophy (MD), motor potentials to transcranial and cervical magnetic stimulation (MEPs) were recorded from the right abductor pollicis brevis muscle in 10 patients with MD and in 10 healthy controls. Cortical and cervical latencies, central motor conduction time (CMCT), stimulus threshold intensity and cortical MEP amplitudes expressed both as absolute values and as %M were analysed. MEP cervical latency, absolute or relative amplitude and excitability threshold did not significantly differ in patients and controls. The mean cortical motor latency and CMCT were significantly prolonged in MD patients with respect to normal subjects. Moreover, CMCTs were found to be significantly related to stimulus threshold intensity (P=0.03) and only marginally related to absolute cortical amplitude (P=0.06). These findings are indicative of a central motor delay, also related to decreased excitability of motor neurons, in patients with MD. No correlations were found between individual neurophysiological parameters and age, duration of disease and clinical impairment. Our results suggest that magnetic stimulation studies can detect subclinical dysfunctions of the central motor system in MD patients, as one of the multisystemic manifestations of the disease, rather independent of the primitive muscle damage.     

Cortical relay time for long latency reflexes in patients with definite multiple sclerosis

BACKGROUND: Long latency reflexes (LLR) include afferent sensory, efferent motor and central transcortical pathways. It is supposed that the cortical relay time (CRT) reflects the conduction of central transcortical loop of LLR. Recently, evidence related to the cortical involvement in multiple sclerosis (MS) has been reported in some studies. Our aim was to investigate the CRT alterations in patients with MS. METHODS: Upper extremity motor evoked potentials (MEP), somatosensory evoked potentials (SEP) and LLR were tested in 28 patients with MS and control subjects (n=22). The patients with MS were classified according to the clinical form (relapsing-remitting [R-R] and progressive groups). The MS patients with secondary progressive and primary progressive forms were considered as the "progressive" group. CRT for LLR was calculated by subtracting the peak latency of somatosensory evoked potentials (SEP) and that of motor evoked potentials (MEP) by transcranial magnetic stimulation from the onset latency of the second component of LLR (LLR2) (CRT = LLR2 - [MEP latency + N20 latency]) RESULTS: Cortical relay time was calculated as 7.4 +/- 0.9 ms in control subjects. Cortical relay time was prolonged in patients with MS (11.2 +/- 2.9 ms) (p<0.0001). The latencies of LLR, MEP and SEP were also prolonged in patients with MS. Cortical relay time was not correlated with disease severity and clinical form in contrast to other tests. CONCLUSIONS: Our findings suggested that CRT can be a valuable electrophysiological tool in patients with MS. Involvement of extracortical neural circuits between sensory and motor cortices or cortical involvement due to MS may cause these findings.     

Electrophysiological contribution to understanding the pathophysiology of Friedreich's ataxia

Friedreich's ataxia (FA) is the most frequent early onset recessive ataxia. Ataxia clinical progression is slow, but the majority of patients is wheelchair-bounded before 30 years of age. Some studies suggested that the peripheral sensory neuropathy was a dynamic process that could explain the clinical worsening. In 1983 an electrophysiological and histological study showed peripheral nerve axonal degeneration unrelated to disease duration or severity. A follow-up study confirmed that symptom progression is independent from peripheral neuropathy that is not progressive. A defective developmment of the largest neurons of the dorsal ganglion was supposed. When the unstable GAA expansion in the X25 gene was identified as the responsible mutation in the majority of FA patients, genotype-phenotype correlation studies were performed. A direct correlation between GAA expansion and involvement of peripheral nerve sensory fibers and central somatosensory pathway was shown. Disease duration correlates with pyramidal, auditory and visual pathway involvement, while the GAA expansion size has no effect.     

Multimodality evoked potentials in amyotrophic lateral sclerosis

Visual, brainstem auditory and somatosensory evoked potentials to medial nerve stimulation were recorded in 27 patients affected by amyotrophic lateral sclerosis. VEP N75, P100, N140, N75-P100 latencies and P100 amplitude, BAEP I-III, III-V and I-V interpeak-latencies were within normal limits in all ALS patients. Somatosensory evoked potentials were abnormally delayed in 8 patients: in 3 arms because of a delayed N9-N13 latency, in 9 arms because of a delayed N13-N19 latency.     

Neurotoxic effects of n-hexane on the human central nervous system: evoked potential abnormalities in n-hexane polyneuropathy.

An outbreak of n-hexane polyneuropathy as a result of industrial exposure occurred in printing factories in Taipei area from December 1983 to February 1985. Multimodality evoked potentials study was performed on 22 of the polyneuropathy cases, five of the subclinical cases, and seven of the unaffected workers. The absolute and interpeak latencies of patterned visual evoked potential (pVEP) in both the polyneuropathy and subclinical groups were longer than in the normal controls. The pVEP interpeak amplitude was also decreased in the polyneuropathy cases. Brainstem auditory evoked potentials (BAEP), showed no difference of wave I latency between factory workers and normal controls, but prolongation of the wave I-V interpeak latencies was noted, corresponding with the severity of the polyneuropathy. In somatosensory evoked potentials (SEPs), both the absolute latencies and central conduction time (CCT) were longer in subclinical and polyneuropathy cases than in the unaffected workers and normal controls. From this evoked potentials study, chronic toxic effects of n-hexane on the central nervous system were shown.