多发性硬化周围神经损害的临床与电生理研究

目的研究多发性硬化(MS)患者周围神经损害的临床特点与电生理改变。方法84例MS患者中,对其中16例有周围神经损害的MS患者(PNMS组)和68例无周围神经损害的MS患者(NPNMS组)的临床及电生理资料进行分析。结果本组MS患者中周围神经损害的发生率为19.0%(16/84)。表现为肢体麻木14例(87.5%),肢体无力12例(75.0%),神经根性疼痛4例(25.0%);腱反射减低15例(93.7%),末梢型感觉障碍13例(81.2%),肌萎缩5例(31.2%)。PNMS组患者的平均年龄[(44.6±12.5)岁]、病程[(39.3±18.3)个月]与NPNMS组[(32.2±11.5)岁、(31.6±17.2)个月]比较差异有显著性(P<0.01,P<0.05)。PNMS组以脊髓型MS(11例,68.7%)多见,NPNMS组以脑型MS(49例,72.1%)多见(均P<0.01)。PNMS组运动神经传导速度(MCV)、感觉神经传导速度(SCV)减慢者分别为79.7%、68.7%,F波异常为75.0%;NPNMS组MCV、SCV减慢者分别为7.9%、5.5%,F波异常为5.9%;两组相比差异有极显著性(均P<0.01)。经皮质类固醇等治疗PNMS组除2例无效外,其余患者均随病情好转而恢复。结论MS合并周围神经损害者年龄偏大,病程较长,脊髓型MS多见;MS出现周围神经损害不影响患者的预后;部分临床上无周围神经损害症状的MS患者神经电生理也有异常,神经电生理检查对MS周围神经损害的诊断有重要意义。     

多发性硬化周围神经损害的临床和肌电图研究

目的研究多发性硬化(MS)周围神经损害的临床及肌电图(EMG)特点?方法回顾性分析29例MS患者的临床及EMG检查资料。结果本组18例(62．1％)有周围神经损害的临床表现和/或EMG异常,其中肢体麻木16例(88．9％)、肢体乏力11例(61．1％)、神经根性疼痛5例(27．8％)、自主冲经损害症状3例(16．7％)、饮水呛咳和吞咽困难2例(11．1％);腱反射减低11例(61．1％)、末梢或根型感觉障碍9例(50％)、肌力减低7例(38．9％)、肌萎缩4例(22．2％)、咽反射减低1例(5．6％)．其发病年龄、病程、冲经功能缺损程度及预后与11例不伴周围神经损害的MS患者基本相似、EMG检查发现自发电位4例(13．8％),运动单位电位波幅增高、时限延长8例(27．6％)、运动神经传导速度减慢15例(51．7％)、感觉神经传导速度减慢13例(44．8％)、波幅减低9例(31．1％)、远端潜伏期延长5例(17．2％)、经皮质类固醇等治疗周围神经损害症状除1例双下肢麻木外,其余均随病情好转而恢复。结论部分MS患者伴周围神经损害表现,可随病情好转而恢复;EMG可帮助判断周围神经损害的部位和程度。     

Peripheral sensory abnormalities in patients with multiple sclerosis.

Although multiple sclerosis primarily affects myelin within the central nervous system, both pathologic and physiological studies suggest that mild deficits in peripheral nervous system myelin may be common. To evaluate this question further, we performed near nerve studies on sural nerves of 14 patients with multiple sclerosis. Peak-to-peak amplitude and maximum conduction velocity were normal in 9 of 14 patients, while minimum conduction velocity, or the velocity of the slowest-conducting component of the sensory action potential, was abnormally reduced in 9 patients. In addition, the supernormal period was evaluated for patients and compared with a control sample; multiple sclerosis patients showed a significant reduction in the amplitude of supernormality. Both the reduction in minimum conduction velocity and the alteration in the supernormal period are consistent with a mild defect in peripheral myelin.     

多发性硬化周围神经损害的肌电图及病理研究

目的：探讨多发性硬化（MS）产生周围神经损害的肌电图，病理特点和影响MS累及周围神经的相关因素。方法：33例MS患者，均满足Poser的确定诊断标准，排除其他神经系统疾病，30名正常自愿受试者作为对照，排除周围神经损害的相关因素，两组分别进行运动，感觉神经传导检测，F波潜伏期及出现率，H反射潜伏期检测，腓肠神经活检，光镜及电镜观察周围神经病理变化。结果：（1）33例MS患者中，9例有根性疼痛，3例有手袜套样感觉障碍，6例不对称性肌萎缩，2例有明显的自主神经症状；（2）肌电图显示复合肌肉动作电位波幅降低，正中神经，尺神经感觉运作电位波幅增高，F波及H反射的潜伏期延长，F波出现率降低。MS周围神经损害的程度与神经功能缺损、病程及病变部位有关，神经功能缺损越重，病程越长，胫神经和腓总神经运动传导波幅降低越明显，正中神经、尺神经感觉动作电位波幅增高越明显；脊髓型MS周围神经受损明显高于脑型；（3）6例患者腓肠神经活检，光镜下可见有髓纤维呈不同程度的髓鞘脱失。电镜下以轴索变性为主，髓鞘板层解离及髓球形成。结论：MS是一种以CNS受损为主的脱髓鞘疾病，在部分患者可对同时累及周围神经系统，脱髓鞘改变主要发生在脊神经根，远端轴突可继发轴索损害，肌电图是比较理想的可全面评价MS周围神经损害的临床检测手段，对判断预后有一定的实用价值。     

多发性硬化周围神经病变的电生理评价

目的研究多发性硬化(MS)患者的周围神经病变，并评价神经电生理技术的应用价值。方法采用神经传导速度(NCV)技术检测MS患者周围神经的运动传导速度(MCV)、感觉传导速度(SCV)及其潜伏期；采用运动诱发电位(MEP)检测正中神经和胫神经的潜伏期；采用F波检测正中神经的出现率和传导速度。结果MS患者NCV均不同程度地减慢，MCV的异常率高于SCV，NCV结果提示轴突损害比脱髓鞘显著。MEP测得肘点和腰4点的潜伏期延长，提示正中神经远端和腰骶神经根功能的损害。部分患者F波的出现率降低．提示周围神经根功能异常。结论MS患者存在周围神经病变；综合运用电生理技术可以全面地评价MS周围神经功能。     

Organic solvents and multiple sclerosis: a case-control study

A case-control study of multiple sclerosis (MS) has been carried out in western Norway. The study included 93% of the patients who had clinical onset of MS in the county of Hordaland during the years 1976–86 (N = 155) and 200 controls, marginally matched for age, sex and residence. There was no statistically significant difference between MS patients and controls with regard to exposure to organic solvents, exposure to the combination organic solvents and welding or to organic solvents and other chemical compounds.     

多发性硬化的临床特点与预后的分析

目的分析多发性硬化(MS)的临床特点与预后。方法回顾性分析117例MS患者的临床资料,从临床、影像学等方面对视神经脊髓型(OSMS)和经典MS型(CMS)进行比较。结果117例MS患者中,OSMS型42例(35.9%),CMS型75例(64.1%)。临床表现中,OSMS患者出现肢体无力(88.1%)、感觉缺失(85.7%)、感觉异常(57.1%)、视物模糊(76.2%)、尿便障碍(73.8%)明显多于CMS患者(70.7%、56.0%、20.0%、45.3%及26.7%)(均P<0.05);出现共济失调(7.1%)、复视(0)及构音不清(0)明显少于CMS患者(42.7%、10.7%及16.0%)(P<0.05～0.01)。OSMS患者出现长节段融合性脊髓病变的比例(94.1%)显著多于CMS患者(48.2%)(P<0.01)。脑脊液检查和诱发电位检查结果OSMS组和CMS组差异无统计学意义。发病5年后扩大的残疾状态量表(EDSS)评分OSMS组(2.42±0.90)显著高于CMS组(1.50±0.88)(P<0.05)。在发病的第1年和第3年,年平均复发次数复发缓解型OSMS[(1.92±1.05)次、(1...     

Peripheral neuropathy in hepatitis-related mixed cryoglobulinemia: electrophysiologic follow-up study

A retrospective study was performed on 27 patients with hepatitis C (HCV)-related mixed cryoglobulinemia (purpura, arthralgia, hepatitis, glomerulonephritis, peripheral neuropathy) to assess peripheral nerve involvement during follow-up of up to 8 years. All patients had the same degree of organ/system involvement initially and were clinically evaluated at least annually. All 27 patients received steroids; 15 also received recombinant interferon-alpha 2b (rIFN-alpha 2b). At first examination, neurological signs and electrodiagnostic findings consistent with peripheral neuropathy were found in 20 (74%) and in 24 (88.8%) patients, respectively. Neurological evaluation and electrodiagnostic data at 3 and 8 years revealed worsening of neuropathy, whereas the other manifestations of mixed cryoglobulinemia (MC) were stable. At the last examination, clinical and electrodiagnostic signs of neuropathy were found in 25 patients (92.5%), occurring in 1 of 3 patients with normal initial findings, and worsened in 8. A more severe neuropathy was observed in 3 (25%) of the patients treated with prednisone alone and in 6 (40%) of the patients additionally treated with rIFN-alpha 2b. Our data confirm that in patients with HCV-related MC, peripheral nerve involvement is frequent, is progressive, and does not seem to benefit by addition of rIFN-alpha 2b to steroid treatment.     

Leber's hereditary optic neuropathy associated with a disorder indistinguishable from multiple sclerosis in a male harbouring the mitochondrial DNA 11778 mutation

This report describes a multiple sclerosis (MS)-like disorder in a male patient with Leber's hereditary optic neuropathy (LHON) harbouring the mitochondrial DNA 11778 base pair mutation. Given the population frequencies of MS and LHON, coincidental occurrence is unlikely. Hypothetically the mitochondrial mutation underlying LHON may contribute to presumably immunologically mediated involvement of other myelinated axons in the central nervous system in susceptible individuals, producing a disorder indistinguishable from MS. We recommend that investigation for oligoclonal bands in CSF, evoked potentials and MR brain scan in these patients be supplemented with mitochondrial DNA analysis.     

Peripheral nervous system involvement in multiple sclerosis

Objective: To investigate possible peripheral nervous system (PNS) affection in patients with multiple sclerosis (MS) by standard nerve conduction velocity (SNC) and pain‐related evoked potentials (PREP), a novel electrophysiological method for diagnosing small fibre neuropathy. Background: Former neuropathological and electrophysiological studies demonstrated subtle PNS affection in MS, but routine measurements are mostly normal. Small fibre polyneuropathy is associated with several autoimmune diseases but has not been investigated in MS yet. Design/Methods: Fifty‐four patients with MS (43 relapsing–remitting, 11 secondary progressive MS) underwent SNC of the tibial, sural and peroneal nerve. Twenty‐one patients additionally underwent PREP. Results: SNC abnormalities were observed in 29.6% of all patients and 14.2% of all nerves examined No abnormalities on PREP were found. Conclusions/Relevance: We demonstrated subtle alterations on routine electrophysiological measurements in patients with MS without hints for small fibre pathology.     

Cyclophosphamide in chronic progressive multiple sclerosis: a comparative study

No effective treatment is presently available for progressive multiple sclerosis (MS). Cyclophosphamide (CFX), a cytotoxic immunosuppressive drug widely used in systemic dysimmune diseases, has been proposed for the treatment of multiple sclerosis with different schedules and controversial results. To evaluate the safety and clinical efficacy of CFX, we compared three different treatment schedules in patients with progressive MS: induction followed by bimonthly boosters for one year (17 patients); bimonthly boosters for one year without previous induction (15 patients); and monthly boosters for one year (21 patients). Survival analysis showed that the percentage of stable patients was significantly higher in the first and third treatment schedule groups. Myelotoxicity occurred in patients treated with induction and boosters (Group A). A high incidence of broncopneumonia was observed in patients undergoing the second treatment schedule (Group B). No major effects were observed in patients treated with monthly boosters (Group C). Response to treatment was limited to secondary progressive form. This study suggests that monthly treatment with CFX might be safely administered in progressive MS patients; its clinical efficacy must be confirmed by an appropriately designed clinical trial.     

Brainstem involvement in multiple sclerosis: a clinical and electrophysiological study

ABSTRACT – A major aim of this study was to determine the diagnostic value of 4 electrophysiological tests in MS, and particularly their effectiveness in detecting signs of brainstem involvement. Therefore, auditory brainstem evoked response (ABER), somatosensory evoked response (SSER), blink reflex and electronystagmographic (ENG) investigative methods were applied to a group of 89 patients with definite, probable or possible multiple sclerosis (MS).
The 4 methods yielded interdependent data, especially where the brainstem function was concerned, thus it can be concluded that a single demyelinating lesion may cause a combination of electrophysiological disorders within a small structure such as the brainstem.
ENG recordings were found to reveal the highest number of asymptomatic abnormalities. The combination of ABER and ENG tests revealed electrophysiological disorders in 81% of all patients. The blink reflex and the SSER tests gave hardly supplementary information.     

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.     

Multiple sclerosis in Jordan: a clinical and epidemiological study

Objectives To characterize the clinical, demographic and epidemiological features of multiple sclerosis (MS) in Jordan. Methods Data for consecutive Jordanian patients, fulfilling the McDonald criteria for clinically definite and clinically probable MS, during the time period 2004–2005 were collected and analyzed in the three major referral centers for MS in Jordan. Results We identified a total of 224 patients (165 females, 87%; 59 males, 13%). The mean (±SD) age of onset was 29.3 (±9.6) years, and mean (±SD) duration of illness was 3.9 (±9.3) years. The prevalence of MS in the city of Amman was 39/100,000. The prevalence of MS in Irbid, north Jordan, was 38/100,000. The most frequent presentation was weakness (30.8%), followed by optic neuritis (20.1%), sensory impairment (19.6%), and ataxia (14.3%). A relapsing remitting pattern was identified in 90.2% of patients, the rest being primary and secondary progressive, and one patient had a progressive relapsing course. Family history of MS was found in 9.4% of the cases. About 60% of the patients were using interferon beta. The degree of physical disability was determined using the Expanded Disability Status Scale (EDSS). Younger age of onset, shorter duration of illness, a relapsing remitting pattern, and use of interferon were identified as statistically significant predictors of less disability. Conclusion Jordan is a medium-high risk country for MS, with prevalence higher than what has previously been reported, possibly representing an increase in incidence. Clinical and demographic characteristics are similar to most reports worldwide. Received in revised form: 17 December 2005     

Peripheral nerve conductions in relapsing remitting multiple sclerosis (RRMS) patients

PurposeThe purpose of this study was to investigate with Elektromioneurografija (EMNG) whether there is any affection on peripheral nerves in (RRMS) patients.Material and MethodMotor and sensory nerve conductions were studied in the control group including 33 RRMS patients and 25 healthy individuals. Expanded Disability Status Scale (EDSS) scores, mean annual attack frequency, duration of disease and treatments of RRMS patients were recorded.ResultsThere was a statistically significant (p < 0.05) elongation in motor distal latency of the right peroneal nerve, slowing in the left peroneal nerve conduction velocity, and an elongation in the F-wave response in the RRMS group compared to the control group. It was observed that motor nerve conduction velocities were slower, albeit not statistically significant, and F wave latencies were longer than control group.ConclusionThere are studies in the literature related to the association between MS and peripheral neuropathy. In this study, we found demyelinating type changes, differing significantly from the control group, in motor nerve conductions in RRMS patients. There may be demyelinating type affection in peripheral nervous system with common autoimmune mechanism in MS, a demyelinating disease of the central nervous system.     

Mitochondrial mutations of Leber's hereditary optic neuropathy: a risk factor for multiple sclerosis

Multiple sclerosis (MS) and Leber's hereditary optic neuropathy (LHON) have been found to occur in combination. Based on an extensive literature search and on a clinical analysis of 55 LHON pedigrees (103 patients) and 40 patients with definite MS, this study concludes that the association of LHON and MS is more than a coincidence, and that carrying a primary LHON mutation is a risk factor for developing MS. All three primary LHON mutations occurring in the European and North American populations have been found to be associated with an MS-like syndrome. The neurological characteristics of MS associated with LHON are indistinguishable from those of MS in general, but the severe and bilateral visual symptoms and signs justify considering these patients as a clinical subgroup of MS and screening them for LHON mutations. However, screening LHON patients for MS appears to be more rewarding. Received: 21 July 1999, Received in revised form: 10 January 2000, Accepted: 8 March 2000     

Autonomic involvement in multiple sclerosis: a pupillometric study

To study pupillary autonomic function in multiple sclerosis (MS), we examined 36 subjects with low disability, preserved visual acuity and no recent history (2 years) of optic neuritis or actual visual complaints. Compared to controls, MS patients showed a greater dilatator reaction with darkness and, for the light reflex, a lower amplitude and contraction rate and a greater recovery of pupillary diameter 5 s after the stimulus. Within the MS group, no difference was found comparing patients with or without the following characteristics: nuclear magnetic resonance imaging evidence of midbrain lesions; increased visual evoked potential P100 latency; and a previous history of optic neuritis. No correlation was found between P100 latency, duration of disease and pupillometric parameters. Our results indicate that in MS patients there is autonomic dysfunction with a reduction of parasympathetic tone and a relative increase in sympathetic dilatator tone to the pupils. We suggest that pupillary abnormalities could be due to non-specific impairment of the central pathways subserving pupil functions.     

Lymphocytapheresis therapy in multiple sclerosis, a preliminary study

10 patients with definite multiple sclerosis (MS), most of them with the chronic progressive type, were treated with lymphocytapheresis during one year. The DSS (Disability Status Scale) improved in 4 patients during the intensive phase of treatment, remained stable in 5 patients and deteriorated in one. The NSS (Neurological Status Scale) showed a stabilisation for the majority of the neurologic functions. Using the ISS (Incapacity Status Scale), an improvement was measured in 6 patients, a stabilisation in one and a deterioration in 3. Exacerbations could not be prevented by lymphocytapheresis.     

T cell vaccination in multiple sclerosis: results of a preliminary study

Myelin basic protein (MBP)-reactive T cells are potentially involved in the pathogenesis of multiple sclerosis (MS), and can be depleted by subcutaneous inoculations with irradiated autologous MBP-reactive T cells (T cell vaccination). This preliminary open label study was undertaken to evaluate whether depletion of MBP-reactive T cells would be clinically beneficial to patients with MS. Fifty-four patients with relapsing-remitting (RR) MS (n=28) or secondary progressive (SP) MS (n=26) were immunized with irradiated autologous MBP-reactive T cells and monitored for changes in rate of relapse, expanded disability scale score (EDSS) and MRI lesion activity over a period of 24 months. Depletion of MBP-reactive T cells correlated with a reduction (40 %) in rate of relapse in RR-MS patients as compared with the pre-treatment rate in the same cohort. However, the reduction in EDSS was minimal in RR-MS patients while the EDSS was slightly increased in SP-MS patients over a period of 24 months. Serial semi-quantitative MRI examinations suggest stabilization in lesion activity as compared with baseline MRI. The findings suggest some potential clinical benefit of T cell vaccination in MS and encourage further investigations to evaluate the treatment efficacy of T cell vaccination in controlled trials. Received: 27 November 2000, Received in revised form: 10 May 2001, Accepted: 11 June 2001     

Peripheral neuropathy as the presenting feature of multiple system atrophy

The authors report a case of multiple system atrophy (MSA) with an onset as a peripheral nerve involvement. Their patient, a 55-year-old man, had a 3-year history of distal weakness and atrophy in upper limbs with dysesthesia in the feet. Other identifiable causes of peripheral neuropathy were ruled out. The authors postulate that peripheral nervous system impairment can anticipate the typical appearance of MSA, and they suggest that, in peripheral neuropathies with autonomic system dysfunction, after excluding main causes of autonomic neuropathy, MSA may need to be suspected.