We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intraveno...We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intravenous immunoglobulin (IVIg) and steroid pulse therapy, which were effective for over 10 years. Her clinical course and laboratory tests were consistent with lower motor neuron syndrome (LMNS) with localized proximal muscle weakness. We suggest that some patients diagnosed as LMNS may remain responsive to IVIg or steroid pulse therapy for a long time.展开更多
Multifocal motor neuropathy(MMN)is the one of the most common acquired immune-mediated inflammatory disorders of the peripheral nervous system.The diagnosis is based on the distribution pattern of the neurological sem...Multifocal motor neuropathy(MMN)is the one of the most common acquired immune-mediated inflammatory disorders of the peripheral nervous system.The diagnosis is based on the distribution pattern of the neurological semiology and the pathological changes of nerve conduction studies(NCS)in classical cases.However,in cases with subtle clinical presentation,an extended diagnostic workup may be needed,such as cerebrospinal fluid examination,laboratory tests,and nerve biopsy.NCS remain nowadays fundamental not only for the diagnosis,but also for the follow-up and measurement of response to immune-treatment in MMN.New challenges arose though,on how best to acquire a static and dynamic imaging of the peripheral nerves,aiming to provide a holistic approach to the nerve impairment.According to the literature,neuromuscular ultrasound is able to detect in MMN patients thickened or swollen cervical roots,peripheral nerves or brachial plexus,findings that suggest ongoing inflammation.This review provides a timely update on the nerve ultrasound findings in MMN.展开更多
目的：分析多灶运动神经病（multifocal motor neuropathy，MMN）的临床及神经电生理特点。方法：对13例MMN患者进行神经传导和常规肌电图（EMG）检查，采用微移（inching）技术判定是否有运动神经传导阻滞（CB）。结果：全部患者均隐...目的：分析多灶运动神经病（multifocal motor neuropathy，MMN）的临床及神经电生理特点。方法：对13例MMN患者进行神经传导和常规肌电图（EMG）检查，采用微移（inching）技术判定是否有运动神经传导阻滞（CB）。结果：全部患者均隐袭起病，进展缓慢，临床上以单肢无力为首发症状，随病情发展，受累部位增加。常规神经传导检查可见所有明显萎缩肌肉的复合肌动作电位（CMAP）波幅下降；13例患者神经受累区域以下所支配肌肉EMG检查可见不同程度的神经原性损害。采用微移技术共测定出CB25处和拟诊CB6处。在3条临床及EMG测定均正常的神经发现5处CB，其余26处CB或拟诊CB均发生于轻度至中度无力的肌肉所对应的20条神经。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。展开更多
目的探讨多灶性运动神经病(multifocal motor neuropathy,MMN)临床表现及电生理特点。方法回顾性分析11例MMN患者的临床表现及电生理结果。结果 11例患者中,男8例,女3例,均为缓慢进展的非对称起病,远端重于近端,伴有肌肉萎缩9例,主观感...目的探讨多灶性运动神经病(multifocal motor neuropathy,MMN)临床表现及电生理特点。方法回顾性分析11例MMN患者的临床表现及电生理结果。结果 11例患者中,男8例,女3例,均为缓慢进展的非对称起病,远端重于近端,伴有肌肉萎缩9例,主观感觉障碍2例,腱反射减弱10例。电生理检查示:正中神经、尺神经等运动神经的持续性多灶性传导阻滞是MMN患者的特征性改变。结论神经电生理检查有助于MMN的早期诊断,利于疾病的早期治疗及预后。展开更多
Background:Multifocal motor neuropathy(MMN),Lewis-Sumner syndrome(LSS),and many chronic inflammatory demyelinating polyradiculoneuropathies(CIDPs)are representative of acquired multifocal polyneuropathy and are charac...Background:Multifocal motor neuropathy(MMN),Lewis-Sumner syndrome(LSS),and many chronic inflammatory demyelinating polyradiculoneuropathies(CIDPs)are representative of acquired multifocal polyneuropathy and are characterized by conduction block(CB).This retrospective study aimed to investigate the demyelinating distribution and the selective vulnerability of MMN,LSS,and CIDP with CB(CIDP-CB)in nerves.Methods:Fifteen LSS subjects(107 nerves),24 MMN subjects(176 nerves),and 17 CIDP-CB subjects(110 nerves)were included.Their clinical information was recorded,blood and cerebrospinal fluid tests were conducted,and nerve conductions of the median,ulnar,radial,peroneal,and tibial nerves were evaluated.CB,temporal dispersion,distal motor latency(DML),and F-wave latency were recorded,and nerve conduction velocity,terminal latency index,and modified F-wave ratio were calculated.Results:CB was more likely to occur around the elbow in CIDP-CB than in MMN(78.6%vs.6.8%,P<0.01)but less likely to occur between the wrist and the elbow than in LSS(10.7%vs.39.3%,P<0.05).Tibial nerve CB was most frequently observed in MMN(47.4%,P<0.05).CIDP-CB was characterized by a prolonged DML in all nerves,and slow motor nerve velocity of the upper limb was significant when CB nerves were excluded(P<0.05).Conclusions:We report the different distributions of segmental and diffuse demyelination of the ulnar and tibial nerves in LSS,MMN,and CIDP-CB.These distinct distributions could help in differentiating among these conditions.展开更多
文摘We report a 50-year-old woman who developed localized proximal muscle weakness, in addition to transient elevation of antibodies to GM-1 ganglioside, without multifocal conduction block. She was treated with intravenous immunoglobulin (IVIg) and steroid pulse therapy, which were effective for over 10 years. Her clinical course and laboratory tests were consistent with lower motor neuron syndrome (LMNS) with localized proximal muscle weakness. We suggest that some patients diagnosed as LMNS may remain responsive to IVIg or steroid pulse therapy for a long time.
文摘Multifocal motor neuropathy(MMN)is the one of the most common acquired immune-mediated inflammatory disorders of the peripheral nervous system.The diagnosis is based on the distribution pattern of the neurological semiology and the pathological changes of nerve conduction studies(NCS)in classical cases.However,in cases with subtle clinical presentation,an extended diagnostic workup may be needed,such as cerebrospinal fluid examination,laboratory tests,and nerve biopsy.NCS remain nowadays fundamental not only for the diagnosis,but also for the follow-up and measurement of response to immune-treatment in MMN.New challenges arose though,on how best to acquire a static and dynamic imaging of the peripheral nerves,aiming to provide a holistic approach to the nerve impairment.According to the literature,neuromuscular ultrasound is able to detect in MMN patients thickened or swollen cervical roots,peripheral nerves or brachial plexus,findings that suggest ongoing inflammation.This review provides a timely update on the nerve ultrasound findings in MMN.
文摘目的：分析多灶运动神经病（multifocal motor neuropathy，MMN）的临床及神经电生理特点。方法：对13例MMN患者进行神经传导和常规肌电图（EMG）检查，采用微移（inching）技术判定是否有运动神经传导阻滞（CB）。结果：全部患者均隐袭起病，进展缓慢，临床上以单肢无力为首发症状，随病情发展，受累部位增加。常规神经传导检查可见所有明显萎缩肌肉的复合肌动作电位（CMAP）波幅下降；13例患者神经受累区域以下所支配肌肉EMG检查可见不同程度的神经原性损害。采用微移技术共测定出CB25处和拟诊CB6处。在3条临床及EMG测定均正常的神经发现5处CB，其余26处CB或拟诊CB均发生于轻度至中度无力的肌肉所对应的20条神经。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。
文摘目的探讨多灶性运动神经病(multifocal motor neuropathy,MMN)临床表现及电生理特点。方法回顾性分析11例MMN患者的临床表现及电生理结果。结果 11例患者中,男8例,女3例,均为缓慢进展的非对称起病,远端重于近端,伴有肌肉萎缩9例,主观感觉障碍2例,腱反射减弱10例。电生理检查示:正中神经、尺神经等运动神经的持续性多灶性传导阻滞是MMN患者的特征性改变。结论神经电生理检查有助于MMN的早期诊断,利于疾病的早期治疗及预后。
基金the Specific Clinical Program of Integrated Traditional Chinese and Western Medicine(Shanghai Municipal Health Commission,China,2017,ID:ZHYY-ZXJHZX-1-201701)。
文摘Background:Multifocal motor neuropathy(MMN),Lewis-Sumner syndrome(LSS),and many chronic inflammatory demyelinating polyradiculoneuropathies(CIDPs)are representative of acquired multifocal polyneuropathy and are characterized by conduction block(CB).This retrospective study aimed to investigate the demyelinating distribution and the selective vulnerability of MMN,LSS,and CIDP with CB(CIDP-CB)in nerves.Methods:Fifteen LSS subjects(107 nerves),24 MMN subjects(176 nerves),and 17 CIDP-CB subjects(110 nerves)were included.Their clinical information was recorded,blood and cerebrospinal fluid tests were conducted,and nerve conductions of the median,ulnar,radial,peroneal,and tibial nerves were evaluated.CB,temporal dispersion,distal motor latency(DML),and F-wave latency were recorded,and nerve conduction velocity,terminal latency index,and modified F-wave ratio were calculated.Results:CB was more likely to occur around the elbow in CIDP-CB than in MMN(78.6%vs.6.8%,P<0.01)but less likely to occur between the wrist and the elbow than in LSS(10.7%vs.39.3%,P<0.05).Tibial nerve CB was most frequently observed in MMN(47.4%,P<0.05).CIDP-CB was characterized by a prolonged DML in all nerves,and slow motor nerve velocity of the upper limb was significant when CB nerves were excluded(P<0.05).Conclusions:We report the different distributions of segmental and diffuse demyelination of the ulnar and tibial nerves in LSS,MMN,and CIDP-CB.These distinct distributions could help in differentiating among these conditions.
