Hostname: page-component-cb9f654ff-mwwwr Total loading time: 0 Render date: 2025-08-07T02:06:48.713Z Has data issue: false hasContentIssue false

Nerve Ultrasound of Multifocal Motor Neuropathy and Motor and Typical Chronic Inflammatory Demyelinating Neuropathy

Published online by Cambridge University Press:  09 June 2025

Jingwen Niu
Affiliation:
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China
Qingyun Ding
Affiliation:
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China
Nan Hu
Affiliation:
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China
Liying Cui
Affiliation:
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China
Mingsheng Liu*
Affiliation:
Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Dongcheng-qu, Beijing, China
*
Corresponding author: Mingsheng Liu; Email: liumingsheng_pumch@163.com

Abstract

Objective:

Both multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyneuropathy (CIDP) are chronic progressive immune-mediated peripheral neuropathies without sensory loss. We aimed to explore the different features of ultrasonographic and electrophysiological changes among MMN, motor CIDP and typical CIDP patients.

Methods:

Nerve ultrasonographic studies were performed in 19 patients with MMN, 15 patients with motor CIDP and 117 patients with typical CIDP. Cross-sectional areas (CSAs) were measured on the bilateral median and ulnar nerves and brachial plexus. Nerve conduction studies (NCSs) were performed on the median and ulnar nerves.

Results:

In patients with MMN and typical CIDP, the percentage enlargement in the brachial plexus (MMN 45.7%, typical CIDP 78%) was similar to that in the arm (MMN 42.9%, typical CIDP 76.8%) and forearm (MMN 42.9%, typical CIDP 79.4%). However, in patients with motor CIDP, the percentage enlargement in the brachial plexus (74.1%) was more pronounced than in the arm (65.5%) and forearm (58.6%). The CMAPerb/CMAPaxilla in MMN was significantly higher than in motor CIDP (median nerve, 0.82 ± 0.28 for MMN and 0.60 ± 0.37 for motor CIDP, P = 0.017). The CSA decreased in the order of typical CIDP, motor predominant CIDP (MPred-CIDP), pure motor CIDP (PM-CIDP) and MMN. The motor nerve conduction velocity increased in the order of typical CIDP, MPred-CIDP, PM-CIDP and MMN. A total of 3/6 PM-CIDP and 3/3 MPred-CIDP patients responded to steroid treatment.

Conclusion:

Treatment response, nerve ultrasonography and NCS in MMN, PM-CIDP, MPred-CIDP and typical CIDP constitute a spectrum.

Résumé

RÉSUMÉ

Échographie des nerfs dans le cas de la neuropathie motrice multifocale et de la neuropathie démyélinisante inflammatoire chronique typique et motrice.

Objectif :

La neuropathie motrice multifocale (NMM) et la polyneuropathie inflammatoire démyélinisante chronique (PIDC) sont des neuropathies périphériques chroniques progressives à médiation immunitaire et sans perte sensorielle. Nous avons ainsi cherché à explorer les différentes caractéristiques des changements échographiques et électrophysiologiques chez des patients atteints de NMM, de PIDC motrice et de PIDC typique.

Méthodes :

Des études par échographie des nerfs ont été effectuées chez 19 patients atteints de NMM, 15 patients atteints de PIDC motrice et 117 patients atteints de PIDC typique. Les surfaces de section transversale (SST) ont été mesurées sur les nerfs médian et ulnaire bilatéraux et sur le plexus brachial. Des études de conduction nerveuse (ECN) ont été menées sur les nerfs médian et cubital.

Résultats :

Chez les patients atteints de NMM et de PIDC typique, le pourcentage d’hypertrophie du plexus brachial (NMM 45,7 %, PIDC typique 78 %) était similaire à celui du bras (NMM 42,9 %, PIDC typique 76,8 %) et de l’avant-bras (NMM 42,9 %, PIDC typique 79,4 %). En revanche, chez les patients atteints de PIDC motrice, le pourcentage d’hypertrophie du plexus brachial (74,1 %) est plus prononcé que celui du bras (65,5 %) et de l’avant-bras (58,6 %). La CMAPerb/CMAPaxilla dans la NMM était significativement plus élevée que dans la PIDC motrice (nerf médian : 0,82 ± 0,28 pour la NMM et 0,60 ± 0,37 pour la PIDC motrice, p = 0,017). En ce qui concerne les SST, on a observé les diminutions les plus notables dans l’ordre suivant : PIDC typique, PIDC à prédominance motrice, PIDC purement motrice et NMM. La vitesse de conduction du nerf moteur a par ailleurs augmenté dans l’ordre suivant : PIDC typique, PIDC à prédominance motrice, PIDC purement motrice et NMM. Au total, 3 patients sur 6 atteints de PIDC purement motrice et 3 patients sur 3 patients atteints de PIDC à prédominance motrice ont répondu à des traitements par stéroïdes.

Conclusion :

La réponse aux traitements, l’échographie des nerfs et les ECN dans le cas de la NMM, de la PIDC purement motrice, de la PIDC à prédominance motrice et de la PIDC typique constituent un spectre (spectrum).

Information

Type
Original Article
Copyright
© The Author(s), 2025. Published by Cambridge University Press on behalf of Canadian Neurological Sciences Federation

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

Van den Bergh, PYK, van Doorn, PA, Hadden, RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. Eur. J. Neurol. 2021;28(11):35563583. DOI: 10.1111/ene.14959.Google Scholar
Van Asseldonk, JT, Franssen, H, Van den Berg-Vos, RM, Wokke, JH, Van den Berg, LH. Multifocal motor neuropathy. Lancet. Neurol. 2005;4(5):309319. DOI: 10.1016/s1474-4422(05)70074-0.Google Scholar
Pegat, A, Boisseau, W, Maisonobe, T, et al. Motor chronic inflammatory demyelinating polyneuropathy (CIDP) in 17 patients: clinical characteristics, electrophysiological study, and response to treatment. J. Peripher. Nerv. Syst. 2020;25(2):162170. DOI: 10.1111/jns.12380.Google Scholar
Doneddu, PE, Cocito, D, Manganelli, F, et al. A typical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J. Neurol. Neurosurg. Psychiatry. 2019;90(2):125132. DOI: 10.1136/jnnp-2018-318714.Google Scholar
Kimura, A, Sakurai, T, Koumura, A, et al. Motor-dominant chronic inflammatory demyelinating polyneuropathy. J. Neurol. 2010;257(4):621629. DOI: 10.1007/s00415-009-5386-x.Google Scholar
Sabatelli, M, Madia, F, Mignogna, T, Lippi, G, Quaranta, L, Tonali, P. Pure motor chronic inflammatory demyelinating polyneuropathy. J. Neurol. 2001;248(9):772777. DOI: 10.1007/s004150170093.Google Scholar
Zhou, XJ, Zhu, Y, Zhu, DS, et al. Different distributions of nerve demyelination in chronic acquired multifocal polyneuropathies. Chin. Med. J. (Engl). 2020;133(21):25582564. DOI: 10.1097/cm9.0000000000001073.Google Scholar
Li, Y, Niu, J, Liu, T, et al. Motor conduction block and conduction velocity in Lewis–Sumner syndrome and multifocal motor neuropathy. J. Clin. Neurosci. 2019;67:1013. DOI: 10.1016/j.jocn.2019.06.044.Google Scholar
Merola, A, Rosso, M, Romagnolo, A, Peci, E, Cocito, D. Peripheral nerve ultrasonography in chronic inflammatory demyelinating polyradiculoneuropathy and multifocal motor neuropathy: correlations with clinical and neurophysiological data. Neurol. Res. Int. 2016;2016:19. DOI: 10.1155/2016/9478593.Google Scholar
Kerasnoudis, A, Pitarokoili, K, Gold, R, Yoon, MS. Bochum ultrasound score allows distinction of chronic inflammatory from multifocal acquired demyelinating polyneuropathies. J. Neurol. Sci. 2015;348(1–2):211215. DOI: 10.1016/j.jns.2014.12.010.Google Scholar
Niu, J, Zhang, L, Hu, N, Cui, L, Liu, M. The distribution pattern of nerve enlargement in clinical subtypes of chronic inflammatory demyelinating polyneuropathy. J. Neuroimaging. 2024;34(1):127137. DOI: 10.1111/jon.13162.Google Scholar
Joint Task Force of the EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J. Peripher. Nerv. Syst. 2010;15(4):295301. DOI: 10.1111/j.1529-8027.2010.00290.x.Google Scholar
Niu, J, Li, Y, Zhang, L, Ding, Q, Cui, L, Liu, M. Cross-sectional area reference values for sonography of nerves in the upper extremities. Muscle Nerve. 2020;61(3):338346. DOI: 10.1002/mus.26781.Google Scholar
Graham, RC, Hughes, RA. A modified peripheral neuropathy scale: the overall neuropathy limitations scale. J. Neurol. Neurosurg. Psychiatry. 2006;77(8):973976. DOI: 10.1136/jnnp.2005.081547.Google Scholar
Niu, J, Li, Y, Liu, T, et al. Serial nerve ultrasound and motor nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2019;60(3):254262. DOI: 10.1002/mus.26611.Google Scholar
Ding, Q, Li, J, Guan, Y, et al. Nerve ultrasound studies in POEMS syndrome. Muscle Nerve. 2021;63(5):758764. DOI: 10.1002/mus.27209.Google Scholar
Niu, J, Zhang, L, Ding, Q, et al. Vagus nerve ultrasound in chronic inflammatory demyelinating polyradiculoneuropathy and Charcot-Marie-Tooth disease type 1A. J. Neuroimaging. 2020;30(6):910916. DOI: 10.1111/jon.12747.Google Scholar
Walter, U, Sobiella, G, Prudlo, J, et al. Ultrasonic detection of vagus, accessory, and phrenic nerve atrophy in amyotrophic lateral sclerosis: relation to impairment and mortality. Eur. J. Neurol. 2024;31(2):e16127. DOI: 10.1111/ene.16127.Google Scholar
Niu, J, Zhang, L, Ding, Q, et al. Reference values for lower limb nerve ultrasound and its diagnostic sensitivity. J. Clin. Neurosci. 2021;86:276283. DOI: 10.1016/j.jocn.2021.01.013.Google Scholar
Padua, L, Granata, G, Sabatelli, M, et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin. Neurophysiol. 2014;125(1):160165. DOI: 10.1016/j.clinph.2013.07.023.Google Scholar
American Association of Electrodiagnostic M, Olney RK. Guidelines in electrodiagnostic medicine. Consensus criteria for the diagnosis of partial conduction block. Muscle. Nerve. Suppl. 1999;8:S225229.Google Scholar
Taylor, BV, Dyck, PJ, Engelstad, J, Gruener, G, Grant, I, Dyck, PJ. Multifocal motor neuropathy: pathologic alterations at the site of conduction block. J. Neuropathol. Exp. Neurol. 2004;63(2):129137. DOI: 10.1093/jnen/63.2.129.Google Scholar
Yeh, WZ, Dyck, PJ, van den Berg, LH, Kiernan, MC, Taylor, BV. Multifocal motor neuropathy: controversies and priorities. J. Neurol. Neurosurg. Psychiatry. 2020;91(2):140148. DOI: 10.1136/jnnp-2019-321532.Google Scholar
Mathey, EK, Park, SB, Hughes, RA, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J. Neurol. Neurosurg. Psychiatry. 2015;86(9):973985. DOI: 10.1136/jnnp-2014-309697.Google Scholar
Busby, M, Donaghy, M. Chronic dysimmune neuropathy: a subclassification based upon the clinical features of 102 patients. J. Neurol. 2003;250(6):714724. DOI: 10.1007/s00415-003-1068-2.Google Scholar
Rattay, TW, Winter, N, Decard, BF, et al. Nerve ultrasound as follow-up tool in treated multifocal motor neuropathy. Eur. J. Neurol. 2017;24(9):11251134. DOI: 10.1111/ene.13344.Google Scholar
Supplementary material: File

Niu et al. supplementary material 1

Niu et al. supplementary material
Download Niu et al. supplementary material 1(File)
File 1.9 MB
Supplementary material: File

Niu et al. supplementary material 2

Niu et al. supplementary material
Download Niu et al. supplementary material 2(File)
File 18.1 KB
Supplementary material: File

Niu et al. supplementary material 3

Niu et al. supplementary material
Download Niu et al. supplementary material 3(File)
File 18.7 KB