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
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.
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.
