多灶性运动神经病的临床特征及治疗

目的:分析多灶性运动神经病(MMN)的临床特征及治疗方法。方法:回顾性分析24例多灶性运动神经病患者的临床资料。结果:24例MMN表现为进行性、非对称性肢体运动无力,以远端为重,伴或不伴感觉障碍。部分患者抗-GM1抗体滴度升高。10例应用激素治疗,8例无效;20例应用免疫球蛋白(IVIg)治疗,15例有效。结论:MMN是一种以运动神经受累为主的不对称性周围神经病,临床症状和体征是诊断MMN的基础,免疫学检查发现抗-GM1抗体滴度升高支持诊断,但与临床症状和体征并非完全相关,免疫球蛋白(IVIg)治疗有效。     

癫癎和热惊厥患儿血清抗心磷脂抗体变化及临床意义

目的 探讨癫(癎)和热惊厥患儿血清抗心磷脂抗体的变化及临床意义.方法 采用酶联免疫吸附试验(ELISA)检测39例癫(癎)患儿,45例热惊厥患儿及40例健康对照组血清中抗心磷脂抗体阳性率水平.结果 热惊厥组和癫(癎)组血清ACA-IgG阳性率均高于对照组,癫(癎)组血清ACA-IgG阳性率高于热惊厥组,差异具有统计学意义.结论 检测热惊厥和癫(癎)患儿血清中抗心磷脂抗体的变化对指导临床诊断、治疗和预防具有一定意义.     

癫(癇)患者血清和脑脊液中抗心磷脂抗体的测定及临床意义

目的 探讨癫(癇)患者血清和脑脊液中抗心磷脂抗体的测定及临床意义.方法 采用酶联免疫吸附试验(ELISA法)检测45例癫(癇)患者及43例健康人群血清和脑脊液中抗心磷脂抗体水平.结果 癫(癇)组血清和脑脊液中抗心磷脂抗体均明显高于健康对照组(χ2=36.42、35.73,P<0.01).结论 癫(癇)发作时患者血清和脑脊液中抗心磷脂抗体水平增加,癫(癇)患者存在自身免疫功能的紊乱.     

多发性肌炎合并间质性肺病17例分析

目的:通过对17例多发性肌炎合并间质性肺病患者临床资料的分析以探讨其临床特点。方法:采用回顾性研究方法。结果:多发性肌炎合并间质性肺病者发热、咳嗽咳痰及关节痛出现率高,血沉明显增快,血抗核抗体(ANA)及抗Jo-1抗体的检出率高,肺部有特殊影像学改变,激素及免疫抑制剂有效。有半数以间质性肺病为首发症状。结论:多发性肌炎合并间质性肺病有特殊的临床表现,治疗效果不差。     

以神经性瘙痒起病的莫旺综合征1例报告及文献复习

莫旺综合征( MoS)是一种男性多见的罕见的自身免疫相关性疾病[1].1890 年,法国内科医生奥古斯汀·玛丽·莫文( Augustin Marie Morvan)首次描述了这种疾病[2].MoS的特征是由于电压门控钾通道( voltage-gated potassium chan-nel,VGKC)抗体导致的中枢、自...     

轻度认知功能障碍老年人的失匹配性负波研究

目的研究轻度认知功能障碍老年人(MCI)失匹配性负波(MMN)的特征.方法应用美国Nicolet BRAVO脑电生理仪,对36例MCI和30例阿尔茨海默病(AD组)及45名正常老人(NC组)的MMN作了检测.结果(1)与正常老年组比较,AD组MMN潜伏期延迟,波幅降低.(2)MCI组无上述变化.(3)AD组MMN潜伏期延迟及波幅低于MCI组.结论MMN技术可作为P300检测的一种补充手段,而用于老年精神科临床.     

老年慢性精神分裂症和Alzheimer病的非匹性负波比较

目的 探讨老年慢性精神分裂症(SCS)和Alzheimer病(AD)在非匹性负波(MMN)检测中的特征.方法 应用美国Nicolet BRAVO脑电生理仪,对38例SCS和32例Alzheimer病(AD组)及40例正常老人(NC组)的MMN作检测.结果(1)与正常老年组比较,AD组MMN潜伏期延迟,波幅降低;(2)SCS组无上述变化;(3)AD组MMN潜伏期延迟及波幅低于SCS组.结论 MMN技术可作为P300检测的一种补充手段,而用于老年精神科临床.     

重复经颅磁刺激对精神分裂症患者事件相关电位的影响

目的:探讨重复经颅磁刺激(rTMS)对精神分裂症患者失匹配负波(MMN)及P300的影响。方法:应用美国脑电生理仪器,对78例精神分裂症患者在rTMS治疗前后进行P300和MMN检测,观察rTMS治疗前后P300和MMN的变化。结果:与正常组比较,精神分裂症组MMN潜伏期延迟,和波幅降低(P0.05或P0.01),P300中的靶波幅P3降低(P0.05)。患者组经过25次rTMS治疗后MMN波幅及P300靶波幅P3明显提高(P0.05或P0.01)。结论:rTMS治疗可以提高精神分裂症患者事件相关电位的MMN及P3波幅。     

老年期抑郁症和Alzheimer病的失匹性负波研究

目的 探讨老年期抑郁症(SD)和Alzheimer病(AD)患者在失匹性负波(MMN)检测中的特征.方法 应用美国Nicolet BRAVO脑电生理仪,对31例SD和30例AD及33名正常老人(NC组)的MMN进行了检测.结果 与正常老年组比较,AD组MMN潜伏期延迟,波幅降低.而SD组无上述变化.AD组MMN潜伏期延迟及波幅低于SD组.结论 MMN技术可作为P300检测的一种补充手段,用于老年精神科临床.     

双相障碍抑郁发作与单相抑郁症失匹配负波的对照研究

目的:比较双相障碍I型抑郁发作与单相抑郁症失匹配负波(MMN)的差异及其与各自临床特征的相关性。方法:对55例双相障碍I型抑郁发作患者(双相组)、55例单相抑郁患者(单相组)以及50名正常对照者(正常对照组)进行MMN检测;采用大体功能评定量表(GAF)评定各组总体功能水平,汉密尔顿抑郁量表(HAMD)评估患者组病情严重程度。在控制性别、年龄、教育年限后,偏相关分析患者组MMN与临床特征及功能的相关性。结果:患者组较正常对照组GAF评分降低(F=53.96,P0.01);患者组较正常对照组MMN潜伏期延长(F=44.31,P0.01)、MMN波幅降低(F=5.39,P0.01);双相组与单相组MMN潜伏期及MMN波幅差异无统计学意义(P均0.05)。双相组MMN潜伏期与HAMD评分和发作次数呈正相关(r=0.51,0.46;P0.01);MMN波幅与HAMD评分和发作次数呈负相关(r=-0.35,-0.39;P0.01);单相组MMN潜伏期与HAMD评分呈正相关(r=0.52,P0.01),MMN波幅与HAMD评分呈负相关(r=-0.47,P0.01)。结论:双相障碍I型抑郁发作与单相抑郁症患者的异常MMN可能是抑郁发作的状态性标记。     

Multifocal motor neuropathy: current concepts and controversies

Multifocal motor neuropathy (MMN) is now a well-defined purely motor multineuropathy characterized by the presence of multifocal partial motor conduction blocks (CB), frequent association with anti-GM1 IgM antibodies, and usually a good response to high-dose intravenous immunoglobulin (IVIg) therapy. However, several issues remain to be clarified in the diagnosis, pathogenesis, and therapy of this condition including its nosological position and its relation to other chronic dysimmune neuropathies; the degree of CB necessary for the diagnosis of MMN; the existence of an axonal form of MMN; the pathophysiological basis of CB; the pathogenetic role of antiganglioside antibodies; the mechanism of action of IVIg treatments in MMN and the most effective regimen; and the treatment to be used in unresponsive patients. These issues are addressed in this review of the main clinical, electrophysiological, immunological, and therapeutic features of this neuropathy.     

Sensory loss in multifocal motor neuropathy: A clinical and electrophysiological study

Some patients fulfilling the criteria for the diagnosis of multifocal motor neuropathy with conduction block (MMN‐CB) at the onset of disease may subsequently develop a sensory loss associated with electrophysiological sensory abnormalities. The latter could represent an overlap between MMN‐CB and multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy. The objective was to specify the features of MMN‐CB with sensory loss (MMN‐CB‐Se). Five patients in a series of 11 consecutive patients who fulfilled the criteria of the American Association of Neuromuscular and Electrodiagnostic Medicine for MMN‐CB at the first examination and were treated periodically with intravenous immunoglobulin (IVIg) developed sensory loss in the course of the disease. In these five patients we compared the clinical, laboratory, and electrophysiological features found after the development of sensory loss with those at the first examination. The mean time to appearance of objective sensory signs was 7.2 years. In three of the five patients the sensory loss was preceded by intermittent paresthesias in the same nerve territories as the motor involvement. The most frequent electrophysiological abnormality was amplitude reduction of sensory nerve action potentials. There were no bilateral or symmetrical clinical and electrophysiological sensory abnormalities. Anti‐GM1 IgM antibodies were positive in four patients. MMN‐CB‐Se could be an overlap between MMN‐CB and MADSAM. It shares the distribution of the sensory disorders encountered in MADSAM, but it is closer to MMN‐CB on clinical and therapeutic levels. Study of more patients would be useful to classify this subgroup more accurately. Muscle Nerve, 2009     

Fulminant multifocal motor neuropathy: a report of two cases

Multifocal motor neuropathy (MMN) shows stepwise progression over decades. The multifocal weakness usually remains asymmetric, confined to distal limb muscles, while sparing cranial, phrenic, and sensory nerves. One electrophysiological hallmark is partial motor conduction block (CB) at sites not exposed to compression; whether CB is an essential feature remains debatable. High titer of anti-GM1 antibodies is found with figures usually between 40% and 50% of patients. Intravenous immuneglobulin (IVIg) is effective in almost 80%, but plasmapheresis and steroids are not. The condition is reported as lethal exceptionally, mimicking motor neuron diseases (MND). We have studied two patients who failed to respond to treatment and who died with respiratory failure; one of the two had high titer of IgM antibody to the ganglioside GM1. Our cases confirm that great attention should be paid in order to define the borderland between MMN and MND and the entity of their clinical and electrophysiological overlaps.     

Acute-onset multifocal motor neuropathy (AMMN): how we meet the diagnosis

Multifocal motor neuropathy (MMN) usually progresses insidiously with lower motor neuron-type weakness, minimal or no sensory symptoms. Diagnostic criteria include motor conduction block (CB) at sites not exposed to compression or entrapment. CBs may persist or reverse irrespective of clinical outcome. Acute onset with generalized weakness is uncommon. We report four patients who presented acutely areflexia, pure motor deficits without sensory disturbances, multifocal CBs persisting at the same motor nerves on serial electrophysiological studies. Three patients had preceding infections; two showed IgM reactivity against the ganglioside GM1. Intravenous immuneglobulin (IVIg) improved or stabilized symptoms. Patients 2,3,4 receive maintenance therapy with IVIg for years. Acute-onset MMN (AMMN) should be differentiated from other immune-mediated neuropathies such as acute inflammatory polyneuropathy either demyelinating (AIDP) or axonal (AMAN), acute motor conduction block neuropathy (AMCBN), acute-onset chronic inflammatory demyelinating polyneuropathy (CIDP). The correct diagnosis deserves implications for patient long-term treatment and prognosis. Moreover, the authors address the problem of defining the spectrum of MMN particularly in the acute setting.     

Multifocal motor neuropathy: clinical and immunological features and response to IVIg in relation to the presence and degree of motor conduction block

OBJECTIVE: To determine whether patients with clinically typical multifocal motor neuropathy (MMN) with or without definite or probable conduction block (CB) differ in terms of clinical presentation, immunological findings, or response to treatment with intravenous immunoglobulin (IVIg). METHODS: 23 consecutive patients were studied with the typical clinical features of MMN, consisting of a progressive multineuropathic motor impairment with minimal or no sensory loss. In 14 patients, electrophysiological studies disclosed the presence of a definite or probable CB according to the criteria proposed by the American Association of Electrodiagnostic Medicine (AAEM) in at least one motor nerve. Six patients had possible CB, defined as a degree of CB 10% less than that required by the AAEM for probable CB, while no CB was detected in three patients. RESULTS: Patients with possible CB did not differ from those with a definite or probable CB in terms of age at disease onset (mean 38.8 v 38.2 years, respectively), distribution and severity of limb weakness, clinical impairment (mean Rankin score 2.2 in both), and frequency of antiganglioside antibodies (33% v 29%). Patients with possible CB had a longer mean disease duration (9 v 5.9 years, p < 0.05) and a less frequent consistent response to IVIg (67% v 86%) than those with a definite or probable CB. Patients without a detectable CB had a similar frequency of antiganglioside antibodies (33%) but had a longer disease duration (20.3 years), greater impairment (Rankin score 2.7), and more frequent signs of axonal degeneration (41% of examined motor nerves) than patients with CB (13-15%, p < 0.005). Only one patient without detectable CB (33%) consistently improved with IVIg. CONCLUSIONS: Patients with possible CB were clinically and immunologically indistinguishable from those with definite or probable CB, albeit with a slightly less frequent response to IVIg. This finding suggests that failure to fulfil AAEM criteria for CB in patients with otherwise clinically typical MMN should not preclude this diagnosis and consequently a treatment trial with IVIg. Whether the longer duration and greater severity of the disease and more frequent axonal impairment in patients without detectable CB than in those with CB explain their lower response to IVIg remains to be established.     

Differential weakness of finger extensor muscles: A clinical pattern of multifocal motor neuropathy

Introduction: Several studies have suggested that differential weakness in muscles supplied by the same motor nerve supports the diagnosis of multifocal motor neuropathy (MMN). Methods: We describe the clinical, electrophysiological, neuroimaging, and laboratory findings of patients with a lower motor syndrome whose clinical presentation included differential finger extension weakness that we have seen in our neuromuscular clinic. Results: We identified 3 patients with hand weakness and 1 patient with asymmetric weakness of the upper extremity. Conduction blocks (CBs) were identified in 1 patient. Anti‐GM1 immunoglobulin M antibodies were detected in 2 of the 3 patients tested. Only 1 patient responded to intravenous immunoglobulin (IVIg). Rituximab was administered in another patient, but we did not detect a response. Conclusions: We suggest that differential finger extension weakness is a feature that may be seen in MMN, even in the absence of CB or response to IVIg. Muscle Nerve 55 : 433–437, 2017     

High-dose intravenous immunoglobulin therapy improved muscle strength in a patient with multifocal motor neuropathy and antibodies against the glycolipid LK1

We report improvement in muscle strength in a patient with multifocal motor neuropathy (MMN) when given high-dose intravenous immunoglobin (i.v.-Ig) treatment. The patient had asymmetrical limb weakness, atrophy and absent or weak reflexes, but no sensory disturbances. Neurography showed multiple conduction blocks in peripheral motor nerves but no sensory nerve abnormalities. Serum and anti-GM1 antibodies were not found, however, the patient had serum antibodies against the glycolipid LK1, an epitope found both in glycolipid and also in some glycoproteins in peripheral nerve myelin. Muscle strength improved 5 days after i.v.-Ig therapy, and lasted about 10 weeks. Repeated courses of treatment resulted in similar improvement. This is, to our knowledge, the first patient reported with MMN found to have antibodies against the glycolipid LK1.     

Activity-dependent conduction block in multifocal motor neuropathy

Previous studies suggested that activity-dependent conduction block (CB) contributes to weakness in multifocal motor neuropathy (MMN). Obtaining more robust evidence for activity-dependent CB is important because it may be a novel target for treatment strategies. We performed nerve conduction studies in 22 nerve segments of 19 MMN patients, before and immediately after 60 seconds of maximal voluntary contraction (MVC) of the relevant muscle. We employed supramaximal electrical stimulation, excluded nerves with marked axonal loss, and adopted criteria for activity-dependent CB. Per segment, the segmental area ratio [area proximal compound muscle action potential (CMAP)/area distal CMAP] was calculated and, per nerve, total area ratio (area CMAP at Erb's point/area distal CMAP) was obtained. MVC induced no changes in mean area ratios and induced no activity-dependent CB. In segments with CB before MVC, the MVC induced increased duration prolongation. In MMN, MVC induced temporal dispersion but no activity-dependent CB.     

Multifocal motor neuropathy without overt conduction block

One of the defining electrophysiological characteristics of multifocal motor neuropathy (MMN) is focal motor nerve conduction block. We have noted occasional patients with typical clinical features of MMN in whom there is no demonstrable conduction block. Upon review of 5 such cases, we conclude that otherwise typical MMN may present without overt conduction block. This subset of patients does not otherwise differ from patients with MMN in whom this hallmark electrodiagnostic feature is present. © 1998 John Wiley & Sons, Inc. Muscle Nerve 21: 243–245, 1998.     

神经电生理检测对多灶性运动神经病诊断价值的初步研究

目的 探讨神经电生理检查在多灶性运动神经病(MMN)中的诊断价值。方法对16例MMN患者及16名健康对照进行运动神经传导速度和感觉神经传导速度检查，记录刺激引出的复合肌肉动作电位的波幅、波宽、面积、位相和时限，进行对比分析，判定是否有运动神经传导阻滞(CB)或暂时性离散(TD)，并有选择性地进行常规肌电图检查。结果16例患者均可见有一根以上运动神经或至少一根运动神经的一个以上部位出现CB或CD。其中13例双上肢正中神经，尺神经出现CB，3例以正中神经、尺神经的远端出现CB首发，随病情进展出现下肢腓深神经CB。仅有2例感觉神经传导速度稍有减慢，波幅略有降低。16例患者神经受累区域以下所支配肌肉肌电图检查见有神经源性损害。结论MMN是一种以远端神经受累为主的不对称性周围神经病，神经电生理检查对诊断和鉴别诊断．MMN起重要作用，CB是MMN的主要神经电生理表现。