多发性硬化周围神经损伤的病理与临床分析

目的：报道12例多发性硬化（MS）患者周围神经病理检查的异常改变，从中证实MS患者存在周围神经的节段性脱髓鞘病损。方法：12例经肌电图检查发现存在周围神经异常改变的患者行腓肠神经活检及病理学观察。结果：11例标本形态上以脱髓鞘为主，8例可见有髓纤维减少，电镜下显示髓鞘失，有髓纤维再生，形成空泡；神经膜细胞增殖生形成葱头改变；7例可见髓鞘板层松解现象，结论：MS患者不但出现CNS的脱髓鞘病理，而且部分患者同时存在周围神经系统的脱髓鞘病损。     

腊肠体样周围神经病的临床和病理

报告４例经腓肠神经活检病理证实的腊肠体样周围神经病，以提高该病的临床认识和诊断能力。方法局麻下做腓肠神经活检，活检标本分别做ＨＥ和Ｍａｓｓｏｎ染色；髓鞘染色；剥离单神经；半薄和超薄切片，分别在光镜或电镜下观察。结果４例均为男性，１３～２１岁发病，其中３例有家族史，临床特征为反复发生的轻微机械性压迫或牵拉后单神经麻痹。电生理检查显示广泛性周围神经损害，传导速度明显减慢。临床符合遗传性压迫易感性麻痹神经病和痛性臂丛神经病。腓肠神经活检可见少数明显增粗的有髓纤维，直径达２０～２８μｍ，髓鞘增厚，而轴索正常，还可见到薄髓纤维。剥离单神经纤维可见局灶性髓鞘增厚，形似腊肠样。电镜下见到髓鞘板层层数增多，无轴索变性，雪旺细胞和无髓纤维未见明显异常。结论腊肠体样周围神经病有周围神经髓鞘发育缺陷，使周围神经易于损伤，是多发性单神经病的病因之一。     

一氧化二氮滥用所致周围神经病的神经电生理特点

目的探讨一氧化二氮(N_2O;俗称"笑气")滥用导致的中毒性周围神经病的神经电生理特点。方法回顾性分析2015-07—2018-03期间中日友好医院神经科就诊的15例N_2O滥用导致的周围神经病患者的临床表现,实验室检查,头、颈、胸椎MRI及神经传导检查情况。对照组44例(肢带型肌营养不良23例、脂质沉积病5例、低钾性周期性麻痹8例、视神经脊髓炎8例)行神经传导检查,所检神经的神经传导速度和动作电位波幅均在正常范围,并排除周围神经病变。其中作为正中神经对照23例(29条)、尺神经对照20例(22条)、腓神经对照25例(25条)、胫神经对照25例(32条)。结果 15例患者均临床表现为肢体无力和麻木,下肢肌力下降及感觉损害程度均较上肢为著。其中8例患者MRI显示颈段或颈胸段脊髓后索倒"V"型长T2病灶。与对照组比较,病变组正中神经、尺神经、腓神经、胫神经运动传导速度(MCV)平均值分别下降14.1%、11.1%、18.3%、21.5%;远端复合肌肉动作电位(CMAP)波幅分别下降29.6%、19.5%、63.6%、82.5%(均P0.05);与对照组比较,病变组上、下肢感觉传导速度(SCV)减慢、下肢感觉神经动作电位(SNAP)波幅下降(均P0.05)。7条腓神经和3条胫神经复合肌肉动作电位波幅未测出。2例患者存在运动神经传导阻滞。结论 N_2O滥用可导致神经系统损害,以颈胸段脊髓后索病变和周围神经病变多见。周围神经损害可同时累及运动纤维及感觉纤维,包括轴索变性和脱髓鞘,下肢损害程度较上肢为著。运动轴索较感觉轴索更易受损。受累神经可出现运动传导阻滞。     

重度骨质疏松症患者周围神经传导功能的定量分析

目的定量分析重度骨质疏松症患者周围神经传导功能的变化与特点。方法检测重度骨质疏松症患者四肢周围神经的传导功能,并与正常值进行定量对比分析。结果所有患者的检测结果均提示四肢多发性周围神经损害或神经根损害,即运动末端潜伏期延长,运动传导速度和感觉传导速度减慢,复合肌肉运动动作电位和感觉神经动作电位波幅降低,F波潜伏期延长且出现率下降,H反射消失等。结论重度骨质疏松症患者均存在不同程度的周围神经病变,其周围神经存在明显的脱髓鞘改变,也存在明确的轴索变性,而且具有四肢神经远近段同时受累,近段受累甚于远段,下肢受累甚于上肢等特点。     

一氧化碳中毒性周围神经病21例临床分析

目的 探讨一氧化碳(CO)中毒对周围神经的损害。方法 对21例急性CO中毒患者，总结其临床和神经电生理特征。结果 肌电图结果显示：受损的正中神经、尺神经、胫后神经传导速度减慢，动作电位波幅降低。结论 中毒是周围神经疾病的主要病因之一，一氧化碳中毒病例合并周围神经病变并不少见。     

多发性硬化伴肌电图异常10例

目的 分析多发性硬化(MS)伴有周围神经病变时的肌电图改变特点.方法 共对69例MS进行了系统的肌电图测定,测定内容包括感觉神经传导、运动神经传导、F波和同芯针电极肌电图,并对测定结果结合临床进行分析.结果 69例MS患者中10例存在肌电图异常.在所测定的神经中,22.2%(12/54)存在运动和/或感觉传导异常,12.5%(2/16)F波异常;13.8%(8/58)肌肉存在神经源性损害的表现.10例中4例患者分别存在1～2根神经的感觉神经传导波幅下降,严重者甚至引不出波形;3例患者存在1～2根神经的运动传导复合肌肉动作电位波幅下降;1例存在胫后神经感觉运动纤维传导减慢;1例表现为双侧正中神经的F波异常;4例患者存在某一肢体同芯针电极肌电图异常.结论 MS患者肌电图检查所发现的异常,一般为单神经或神经根病变,以轴索丢失为主,其病因较难用MS的发病机制来解释.     

神经活检在周围神经疾病诊断中的意义及与电生理检查相关性分析

目的探讨神经活检在周围神经疾病诊断中的意义,以及神经活检与电生理检查的相关性。方法 12例周围神经病患者均予腓肠神经活检、神经电生理检查,比较二者对轴索、髓鞘损害的诊断情况,统计电生理检查与神经活检诊断的符合率。结果 (1)电生理检查结果检出轴索损害/髓鞘损害9例,神经活检结果发现11例患者伴有髓鞘或轴索损害。(2)神经活检对于周围神经疾病诊断有决定性意义有3例,其余9例也均起到了证实临床诊断的作用。(3)光镜诊断、电镜诊断与电生理检查结果比较无明显差异(P>0.05)。结论神经活检技术对于发现间质改变及亚临床、亚电生理神经损害有明显优势,对于判断周围神经疾病患者的损害类型仍需联合电生理检查进行综合诊断。     

慢性炎性脱髓鞘性多发神经根神经病的肌电图异常特点和诊断价值研究

目的探讨慢性炎性脱髓鞘性多发神经根神经病(CIDP)的肌电图特点和评价病情严重程度的价值。方法收集39例2018年9月至2020年12月收治的CIDP患者的临床资料。采用Hughes残疾评分评估患者病情严重程度。肌电图检查(1)4条运动神经:正中神经、尺神经、胫神经、腓神经的复合肌肉动作电位(CMAP)波幅、运动传导速度(MCV)和F波潜伏期;(2)3条感觉神经:正中神经、尺神经、腓肠神经的感觉神经传导速度(SCV)、动作电位(SNAP)波幅。根据电生理检测结果将患者分为A组(所测神经均可引出波形)和B组(≥1条神经测不出波形)。用Pearson相关性分析MCV、CMAP波幅和F波潜伏期与CIDP病情严重程度的相关性;用受试者工作特征曲线(ROC)评价MCV、CMAP及F波潜伏期对CIDP的诊断价值。结果与A组比较,B组Hughes残疾评分、脑神经受累、肢体无力、腱反射减弱、肌肉萎缩比例,MCV和CMAP波幅、F波潜伏期、SNAP波幅异常率以及F波潜伏期显著增加;而MCV、CMAP波幅均显著降低(均P0.05)。正中神经和尺神经MCV,胫神经CMAP波幅与病情严重程度呈显著负相关;正中神经、尺神经F波潜伏期与病情严重程度呈显著正相关(均P0.05)。ROC曲线分析结果显示,4条运动神经的MCV、CMAP及F波潜伏期均在CIDP的诊断中具有一定价值(均AUC0.07),3项指标联合可显著提高CIDP诊断的敏感度和特异度。结论 CIDP神经电生理表现为MCV明显减慢、CMAP波幅降低和F波潜伏期延长,神经损伤严重的CIDP患者此3项指标异常率更高,联合MCV、CMAP及F波潜伏期3项指标可提高CIDP诊断的敏感度和特异度。     

感觉轴索神经病的临床与神经生理特点

目的探讨感觉轴索神经病的临床和神经生理特点。方法选择24例感觉轴索神经 病患者及88名年龄匹配的健康人,电生理检查用皮肤表面电极超强刺激,表面电极记录,测试尺神 经、正中神经和胫神经不同节段的运动潜伏期、运动传导速度及其CMAP波幅、时限,感觉传导速度及其波幅等。结果22例患者表现为肢体麻木和感觉性共济失调,18例患者远端的痛觉和(或)音叉振动觉减退或消失,17例以下肢为著,所有患者均不伴肌无力和肌束震颤。病因包括肺癌、糖尿病、遗传性脊髓小脑共济失调、酒精中毒和药物中毒等。神经传导显示感觉神经动作电位波幅明显下降,感觉神经传导速度减慢,两者与年龄匹配的健康人相比,差异有非常显著性意义(P<0.001、0.01);运动神经传导速度和波幅两组差异没有显著性意义(P>0.05)。结论感觉轴索神经病是一类多因素造成的临床综合征,以感觉纤维轴索损害为主,临床表现为以下肢为著的感觉症状体征,神经传导检测主要的改变是感觉神经动作电位(SNAP)波幅的减低或消失、伴或不伴感觉传导速度的改变,其常见病因有肿瘤、中毒、代谢性疾病、神经系统遗传病等。     

肌电图在探讨重症监护患者发生多发性神经病早期诊断中的价值,附1例报告

目的探讨肌电图在重症监护病人发生多发性神经病的早期诊断及治疗中的作用。方法床旁肌电图检查,观察患者运动神经传导,针极肌电图,H反射及F波。结果神经电图,针极肌电图检测显示,有轴索及髓鞘纤维变性损害。表现为纤颤,正相电位。运动及感觉传导速度减慢,波幅降低。F波、H波潜伏期延长。结论针极肌电图及神经传导速度测定可以获得神经病变的严重程度和分布范围的完整性,对诊断,治疗提供有价值的信息。     

Peripheral nerves injury of electrophysiology and pathology in MS

OBJECTIVE We report the EMG and pathological features of sural nerve biopsy of 12 patients with MS. The pathological of thesel 1 patients demonstrated demyelination injury of peripheral nerves.METHODS Twelve cases abnomaly are screened with EMG from60 cases MS. Sural nerve biopsy were analyzed by HE AND loyez, and electron microscopy. RESULTS EMG showed slow of MCV in 9 patients and SCV in 7 patients. Myelinated fibers was the presence in 8 sural nerve biopsy patients and most striking demyelinating fibers, regeneration of myelinated fibers. CONCLUSION Ms is characterized by demyelinating disorder limited to the CNS.There are,howeve ,the results of this study sugee that combine with PNS demyelinating injury in MS may be more frequent than is generally assumed.     

Chronic inflammatory demyelinating polyradiculoneuropathy associated with central nervous system involvement--as compared to multiple sclerosis

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with central nervous system (CNS) demyelinating lesions has recently been reported to mimic multiple sclerosis (MS). In this paper, a series of patients with CIDP were examined to see if they had CNS involvement. CIDP patients with CNS lesions were then compared to patients with MS with peripheral nervous system (PNS) involvement for similarities. CNS and PNS involvement were detected by clinical symptoms, neurological findings, neuro-otological and neuro-ophthalmological tests, electrophysiological examinations such as electroencephalography, evoked potentials, blink reflex, conventional peripheral nerve conduction studies and electromyography, as well as computed tomography and magnetic resonance imaging (MRI). There were 7 of 17 CIDP patients with CNS involvement, but only 2 of 59 MS patients with PNS lesions were found. The rate of CIDP with CNS involvement (41.2%) was higher than that of MS with PNS lesions (3.4%). The CNS signs and symptoms of 7 CIDP patients were not so constant as their PNS symptoms, and consisted of 1 case with optic neuritis, 4 cases with cerebellar atxia and/or nystagmus, and 3 cases with spinal symptoms. These signs and symptoms are all well known in MS. Prolonged latencies on evoked potentials and high signal white matter lesions on T2 weighted MRI, indicating demyelinating CNS lesions were also similar to those found in MS. The CNS involvement in those patients with CIDP was therefore similar in character to those found in MS, but was far less severe than the PNS finding.(ABSTRACT TRUNCATED AT 250 WORDS)     

Subclinical lesions of peripheral nervous system in multiple sclerosis patients

BACKGROUND AND PURPOSE: In the last years the presence of peripheral nervous system (PNS) lesions has been noted in patients with multiple sclerosis (MS). The frequency and degree of PNS damage reported by many authors differ among publications, so does the type of PNS lesions. The aim of our study was to perform an electrophysiological evaluation of the peripheral nervous system in patients with a definite diagnosis of multiple sclerosis and without any clinical signs of peripheral neuropathy. MATERIAL AND METHODS: 110 patients were included in the study, comprising 70 people with a definite diagnosis of multiple sclerosis and 40 people without any symptoms of organic nervous system lesion serving as a control group. During neurologic examination of MS patients the degree of disability measured by EDSS scale, the duration of the disease as well as number of relapses were assessed. A "disease progression factor" was calculated by dividing a number of relapses by disease duration in years. Patients with common etiologies for peripheral neuropathy such as diabetes, renal insufficiency, thyroid gland dysfunction, proliferative disorders etc. were excluded from the study. Orthodromic motor conduction and late responses (F wave) in median, ulnar, peroneal and tibial nerves as well as sensory conduction in median, ulnar (orthodromic) and sural (antidromic) nerves were evaluated. RESULTS: There was electrophysiological evidence of peripheral nervous system lesions in at least one nerve in 52 (74.2%) MS patients. In 30 patients (42.8%) more than one peripheral nerve was lesioned. There were more significant differences noted during the examination of sensory nerves. Sensory amplitudes in all of the sensory nerves examined were significantly lower than in control group. Furthermore we observed slow sensory conduction velocities and prolonged sensory latencies in ulnar and sural nerves. There were significant differences between the two groups of patients concerning motor conduction too: prolonged distal latency in tibial and sural nerves, prolonged F wave latency in median, peroneal and tibial nerves, low motor amplitude in ulnar and peroneal nerves, low motor conduction velocity in ulnar nerve -- all noted in MS patients. We found no correlation between conduction parameters and the patients' age, disease duration, number of relapses and disease progression degree. CONCLUSIONS: We found out that subclinical peripheral nervous system abnormalities are very frequent in MS patients. We noted both sensory and motor nerve lesions of a demyelinating-axonal character. Sensory abnormalities were more pronounced than motor ones. There was no correlation between the degree of PNS lesions and the patients' age and/or progression of multiple sclerosis.     

Genotype-phenotype correlation in hereditary motor-sensory neuropathy type IA associated with duplication in chromosome 17p11.2-12

Results of clinical, electrophysiological and morphological examination, were presented in 19 patients from 8 families with hereditary motor-sensory neuropathy type I (HMSN type I) with 17p11.2-12 duplication (i.e. CMT IA). The course of the disease was rather mild, slowly progressive. Generalized demyelinating lesion of peripheral nerves was found on EMG examination, with median nerve conduction velocity between 10-20 m/s and very prolonged F wave latency. Sural nerve biopsy was characteristic of chronic demyelinating process. Phenotypic characteristics of our HMSN type I patients shows clinical, electrophysiological and morphological homogeneity, however there are some data from literature indicating possibility of intrafamilial and interfamilial variability.     

Brucella: a cause of peripheral neuropathy

Brucellosis is a common infectious disease in Mediterranean countries. We evaluated the peripheral nerve involvement in patients with brucellosis. Thirty-eight patients with brucellosis were examined. Four of them were excluded because of B(12) deficiency and diabetes mellitus. Thirty-four patients were included. The average age was 43.08 +/- 15.3 years. Patients were divided into two groups according to the abnormality in their peripheral nervous system (PNS) examination. All patients underwent nerve conduction and needle electromyography EMG studies. Twenty normal healthy subjects were used as a control group. Axonal sensorimotor neuropathy was determined in 12 patients who also had abnormality in PNS examination. After 6 months of treatment, nerve conduction studies were nearly normal in these patients. The EMG findings of the remaining 22 patients were normal, as well as the clinical examination. However, the motor conduction velocities of median (p < 0.001), peroneal (p < 0.001), and ulnar (p < 0.05) nerves were decreased, F wave latencies were prolonged in the posterior tibial and peroneal nerve, and distal latency was also prolonged in the posterior tibial nerve (p < 0.05) when compared to healthy subjects. Sensory conduction velocities of the median (p < 0.001), ulnar and sural (p < 0.05) nerve were also decreased. Brucellosis may be considered as a cause of clinical or subclinical peripheral neuropathy and should be evaluated especially in endemic areas.     

The importance of studying sural nerve conduction and late responses in the evaluation of alcoholic subjects

Motor conduction velocities of median, ulnar, peroneal, and tibial nerves and sensory conduction velocities of median and ulnar nerves were studied in 30 alcoholic subjects and a similar group of control subjects. The results were compared to sural nerve conduction velocities and late response latencies (H reflex, F response). The latter two techniques improved the diagnostic yield by 20%: Whereas 73% of our patients showed an abnormality of conduction with conventional techniques, 93% had an abnormality of sural nerve conduction, late response latencies, or both. Abnormalities of motor and sensory conduction, which were more prominent in the lower limbs than the arms, could be documented in patients who did not have any clinical evidence of peripheral neuropathy. The electrophysiologic studies performed in the present study suggest that "axonal degeneration" is the underlying pathologic process in alcoholic peripheral nerve disease.     

Peripheral nervous system involvement in chronic spinal cord injury

Introduction: Upper motor neuron disorders are believed to leave the peripheral nervous system (PNS) intact. In this study we examined whether there is evidence of PNS involvement in spinal cord injury (SCI). Methods: Twelve subjects with chronic low cervical or thoracic SCI were included prospectively. Needle electromyography was done in 10 different muscles in each subject bilaterally. Nerve conduction studies (NCS) were conducted in the fibular, tibial, and femoral motor and fibular and sural sensory nerves. Results: Half the subjects had widespread abnormal spontaneous activity (SA), and the amount of SA correlated inversely with reflex activity and nerve length. Fibular nerve entrapment across the knee was seen in 6 subjects, and sciatic nerve entrapment was seen in 1. Apart from entrapment neuropathies, NCS changes were found predominantly in motor nerves. Conclusion: The presence of widespread electrophysiologic changes outside entrapment sites indicates that SCI has a significant impact on the entire PNS, affecting the motor part predominantly. Muscle Nerve 52 : 1016–1022, 2015     

Somatosensory evoked potentials after median and tibial nerve stimulation in healthy newborns.

Cortical and spinal somatosensory evoked potentials (SEPs) have been recorded after median and tibial nerve stimulation in healthy newborns. Spinal SEPs were readily obtained and recorded in all but one neonates after stimulation of both nerves. Cortical SEPs were more frequently recorded after median nerve (87%) than after tibial nerve stimulation (73%) but the shape of cortical SEPs obtained after tibial nerve stimulation was less variable. The mean feature of cortical SEPs was a negative wave (N27) for median nerve and a positive wave (P32) for tibial nerve. The present results demonstrate the feasibility of obtaining in the same baby, spinal and cortical SEPs after stimulation of median and tibial nerve, giving information on the functional integrity of central and peripheral somatosensory pathways which supply upper and lower limbs.     

Experimental clioquinol intoxication in rats: Abnormalities in optic nerves and small nerve cells of dorsal root ganglia

Summary Pathological abnormalities in the central (CNS) and peripheral nervous system (PNS) were produced in rats by daily administration of 300–400 mg/kg clioquinol for 7–40 days. The changes comprised axonal degeneration of optic nerve fibers and mitochondrial swelling in small nerve cells of dorsal root ganglia. There were occasional myelin splittings in the spinal nerve roots. No apparent changes were observed in the spinal cord or peripheral nerves. Similar changes were occasionally encountered to a lesser extent in control rats by restricting the diet and water to maintain the body weight comparable to the ranges of experimental animals. It seemed that not only clioquinol intoxication but nutritional deficiency also contributed, in part, to the production of these CNS and PNS abnormalities. The possibility of the presence of peripheral neuropathy in subacute myelooptic neuropathy is discussed.Supported by a grant from the study of subacute myelo-optico neuropathy from the Intractable Disease Division, Public Health Bureau, Ministry of Health and Welfare, Japan