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Progress in the nerve ultrasound of inflammatory peripheral neuropathies

  • Jingwen Niu
  • Mingsheng Liu

Abstract

Objective: In recent years, high-resolution nerve ultrasound has been increasingly used as a complementary tool to nerve conduction studies in the diagnosis of peripheral neuropathies. Inflammatory peripheral neuropathies include a heterogeneous group of neuropathies mainly including chronic inflammatory demyelinating polyneuropathies (CIDP), multifocal motor neuropathy (MMN), systemic vasculitic neuropathy (SVN), and Guillain-Barre syndrome (GBS). We describe the ultrasonic characteristics of different inflammatory neuropathies.

Data sources and study selection: The MEDLINE database was searched to find studies on nerve sonography in inflammatory peripheral neuropathies. Reported studies up to December 2022 were included in this review.

Results: In neuropathies, the changes in ultrasound include enlarged nerve size, nerve echo-intensity, fascicle diameter and vascularity. Most CIDP patients have moderately enlarged CSA, some have dramatically enlarged CSA, and few have CSA within the normal range. Since the enlargement patterns are different, ultrasound is useful to help differentiate CIDP from CMT1. In follow-ups after immune treatment, nerve CSAs in CIDP could decrease, increase, or remain unchanged. Regional enlargement next to normal segments predominated in MMN. Nerve enlargement and hyper vascularization were found in vasculitic neuropathy. In GBS patients, there were enlarged peripheral nerve and nerve roots, which became normal at follow-up.

Conclusions: High-resolution nerve ultrasound is complementary to the diagnosis of inflammatory peripheral neuropathies. CSA enlargement and its distribution are the most useful and quantifiable parameters in the evaluation of peripheral nerves. For patients suspected to have inflammatory neuropathies, we advise measuring the CSA of several pre-determined sites in the median, ulnar nerve, C5-8 cervical roots, and brachial plexus.

Section

References

  1. Collins MP, Dyck PJ, Gronseth GS, Guillevin L, Hadden RD, Heuss D, et al. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst 2010;15(3):176-184.doi:10.1111/j.1529-8027.2010.00281.x
  2. Herraets IJT, Goedee HS, Telleman JA, van Eijk RPA, van Asseldonk JT, Visser LH, et al. Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies. Neurology 2020;94(14):e1470-e1479.doi:10.1212/WNL.0000000000008978
  3. Decard BF, Pham M, Grimm A. Ultrasound and MRI of nerves for monitoring disease activity and treatment effects in chronic dysimmune neuropathies - Current concepts and future directions. Clin Neurophysiol 2018;129(1):155-167.doi:10.1016/j.clinph.2017.10.028
  4. Kramer M, Grimm A, Winter N, Dörner M, Grundmann-Hauser K, Stahl JH, et al. Nerve Ultrasound as Helpful Tool in Polyneuropathies. Diagnostics (Basel) 2021;11(2).doi:10.3390/diagnostics11020211
  5. Burg EW, Bathala L, Visser LH. Difference in normal values of median nerve cross-sectional area between Dutch and Indian subjects. Muscle Nerve 2014;50(1):129-132.doi:10.1002/mus.24124
  6. Cartwright MS, Shin HW, Passmore LV, Walker FO. Ultrasonographic reference values for assessing the normal median nerve in adults. J Neuroimaging 2009;19(1):47-51.doi:10.1111/j.1552-6569.2008.00256.x
  7. Kerasnoudis A, Pitarokoili K, Behrendt V, Gold R, Yoon MS. Cross sectional area reference values for sonography of peripheral nerves and brachial plexus. Clin Neurophysiol 2013;124(9):1881-1888.doi:10.1016/j.clinph.2013.03.007
  8. Boehm J, Scheidl E, Bereczki D, Schelle T, Aranyi Z. High-resolution ultrasonography of peripheral nerves: measurements on 14 nerve segments in 56 healthy subjects and reliability assessments. Ultraschall Med 2014;35(5):459-467.doi:10.1055/s-0033-1356385
  9. Seok HY, Jang JH, Won SJ, Yoon JS, Park KS, Kim BJ. Cross-sectional area reference values of nerves in the lower extremities using ultrasonography. Muscle Nerve 2014;50(4):564-570.doi:10.1002/mus.24209
  10. Won SJ, Kim BJ, Park KS, Yoon JS, Choi H. Reference values for nerve ultrasonography in the upper extremity. Muscle Nerve 2013;47(6):864-871.doi:10.1002/mus.23691
  11. Won SJ, Kim BJ, Park KS, Kim SH, Yoon JS. Measurement of cross-sectional area of cervical roots and brachial plexus trunks. Muscle Nerve 2012;46(5):711-716.doi:10.1002/mus.23503
  12. Bathala L, Kumar P, Kumar K, Shaik A, Visser LH. Normal values of median nerve cross-sectional area obtained by ultrasound along its course in the arm with electrophysiological correlations, in 100 Asian subjects. Muscle Nerve 2014;49(2):284-286.doi:10.1002/mus.23912
  13. Bathala L, Kumar P, Kumar K, Visser LH. Ultrasonographic cross-sectional area normal values of the ulnar nerve along its course in the arm with electrophysiological correlations in 100 Asian subjects. Muscle Nerve 2013;47(5):673-676.doi:10.1002/mus.23639
  14. Sugimoto T, Ochi K, Hosomi N, Mukai T, Ueno H, Takahashi T, et al. Ultrasonographic reference sizes of the median and ulnar nerves and the cervical nerve roots in healthy Japanese adults. Ultrasound Med Biol 2013;39(9):1560-1570.doi:10.1016/j.ultrasmedbio.2013.03.031
  15. Cartwright MS, Shin HW, Passmore LV, Walker FO. Ultrasonographic findings of the normal ulnar nerve in adults. Arch Phys Med Rehabil 2007;88(3):394-396.doi:10.1016/j.apmr.2006.12.020
  16. Yalcin E, Onder B, Akyuz M. Ulnar nerve measurements in healthy individuals to obtain reference values. Rheumatol Int 2013;33(5):1143-1147.doi:10.1007/s00296-012-2527-9
  17. 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):338-346.doi:10.1002/mus.26781
  18. Grimm AS, Schubert C, Grimm A, Stahl JH, Küpper H, Horber V, et al. Normative Observational Nerve Ultrasound Values in School-Age Children and Adolescents and Their Application to Hereditary Neuropathies. Front Neurol 2020;11(303.doi:10.3389/fneur.2020.00303
  19. Granata G, Pazzaglia C, Calandro P, Luigetti M, Martinoli C, Sabatelli M, et al. Ultrasound visualization of nerve morphological alteration at the site of conduction block. Muscle Nerve 2009;40(6):1068-1070.doi:10.1002/mus.21449
  20. Di Pasquale A, Morino S, Loreti S, Bucci E, Vanacore N, Antonini G. Peripheral nerve ultrasound changes in CIDP and correlations with nerve conduction velocity. Neurology 2015;84(8):803-809.doi:10.1212/WNL.0000000000001291
  21. Grimm A, Schubert V, Axer H, Ziemann U. Giant nerves in chronic inflammatory polyradiculoneuropathy. Muscle Nerve 2017;55(2):285-289.doi:10.1002/mus.25272
  22. Hartig F, Ross M, Dammeier NM, Fedtke N, Heiling B, Axer H, et al. Nerve Ultrasound Predicts Treatment Response in Chronic Inflammatory Demyelinating Polyradiculoneuropathy-a Prospective Follow-Up. Neurotherapeutics 2018;15(2):439-451.doi:10.1007/s13311-018-0609-4
  23. Padua L, Granata G, Sabatelli M, Inghilleri M, Lucchetta M, Luigetti M, et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol 2014;125(1):160-165.doi:10.1016/j.clinph.2013.07.023
  24. Niu J, Li Y, Liu T, Ding Q, Cui L, Guan Y, et al. Serial nerve ultrasound and motor nerve conduction studies in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve 2019;60(3):254-262.doi:10.1002/mus.26611
  25. Qrimli M, Ebadi H, Breiner A, Siddiqui H, Alabdali M, Abraham A, et al. Reference values for ultrasonograpy of peripheral nerves. Muscle Nerve 2016;53(4):538-544.doi:10.1002/mus.24888
  26. Tagliafico A, Martinoli C. Reliability of side-to-side sonographic cross-sectional area measurements of upper extremity nerves in healthy volunteers. J Ultrasound Med 2013;32(3):457-462
  27. Simon NG, Talbott J, Chin CT, Kliot M. Peripheral nerve imaging. Handb Clin Neurol 2016;136(811-826.doi:10.1016/b978-0-444-53486-6.00040-5
  28. Zaidman CM, Al-Lozi M, Pestronk A. Peripheral nerve size in normals and patients with polyneuropathy: an ultrasound study. Muscle Nerve 2009;40(6):960-966.doi:10.1002/mus.21431
  29. Grimm A, Vittore D, Schubert V, Lipski C, Heiling B, Decard BF, et al. Ultrasound pattern sum score, homogeneity score and regional nerve enlargement index for differentiation of demyelinating inflammatory and hereditary neuropathies. Clin Neurophysiol 2016;127(7):2618-2624.doi:10.1016/j.clinph.2016.04.009
  30. Zaidman CM, Harms MB, Pestronk A. Ultrasound of inherited vs. acquired demyelinating polyneuropathies. J Neurol 2013;260(12):3115-3121.doi:10.1007/s00415-013-7123-8
  31. Sugimoto T, Ochi K, Hosomi N, Takahashi T, Ueno H, Nakamura T, et al. Ultrasonographic nerve enlargement of the median and ulnar nerves and the cervical nerve roots in patients with demyelinating Charcot-Marie-Tooth disease: distinction from patients with chronic inflammatory demyelinating polyneuropathy. J Neurol 2013;260(10):2580-2587.doi:10.1007/s00415-013-7021-0
  32. Niu J, Zhang L, Fan J, Liu J, Ding Q, Guan Y, et al. Nerve ultrasound may help predicting response to immune treatment in chronic inflammatory demyelinating polyradiculoneuropathy. Neurol Sci 2022.doi:10.1007/s10072-022-05882-7
  33. Fionda L, Di Pasquale A, Morino S, Leonardi L, Vanoli F, Loreti S, et al. Changes of clinical, neurophysiological and nerve ultrasound characteristics in CIDP over time: a 3-year follow-up. J Neurol 2021.doi:10.1007/s00415-021-10485-x
  34. Grimm A, Vittore D, Schubert V, Rasenack M, Decard BF, Heiling B, et al. Ultrasound aspects in therapy-naive CIDP compared to long-term treated CIDP. J Neurol 2016;263(6):1074-1082.doi:10.1007/s00415-016-8100-9
  35. Niu J, Cui L, Liu M. Multiple Sites Ultrasonography of Peripheral Nerves in Differentiating Charcot-Marie-Tooth Type 1A from Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Front Neurol 2017;8(181.doi:10.3389/fneur.2017.00181
  36. Winter N, Vittore D, Gess B, Schulz JB, Grimm A, Dohrn MF. New Keys to Early Diagnosis: Muscle Echogenicity, Nerve Ultrasound Patterns, Electrodiagnostic, and Clinical Parameters in 150 Patients with Hereditary Polyneuropathies. Neurotherapeutics 2021;18(4):2425-2435.doi:10.1007/s13311-021-01141-3
  37. Kerasnoudis A, Pitarokoili K, Behrendt V, Gold R, Yoon MS. Nerve ultrasound score in distinguishing chronic from acute inflammatory demyelinating polyneuropathy. Clin Neurophysiol 2014;125(3):635-641.doi:10.1016/j.clinph.2013.08.014
  38. Grimm A, Oertl H, Auffenberg E, Schubert V, Ruschil C, Axer H, et al. Differentiation Between Guillain-Barré Syndrome and Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuritis-a Prospective Follow-up Study Using Ultrasound and Neurophysiological Measurements. Neurotherapeutics 2019;16(3):838-847.doi:10.1007/s13311-019-00716-5
  39. 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(9478593.doi:10.1155/2016/9478593
  40. Niu J, Ding Q, Fan J, Zhang L, Liu J, Guan Y, et al. Nerve Ultrasound Performances in Differentiating POEMS Syndrome from CIDP. Neurotherapeutics 2022;19(2):455-463.doi:10.1007/s13311-022-01209-8
  41. Omejec G, Zgur T, Podnar S. Diagnostic accuracy of ultrasonographic and nerve conduction studies in ulnar neuropathy at the elbow. Clin Neurophysiol 2015;126(9):1797-1804.doi:10.1016/j.clinph.2014.12.001
  42. Zaidman CM, Pestronk A. Nerve size in chronic inflammatory demyelinating neuropathy varies with disease activity and therapy response over time: a retrospective ultrasound study. Muscle Nerve 2014;50(5):733-738.doi:10.1002/mus.24227
  43. Telleman JA, Herraets IJT, Goedee HS, van Eijk RPA, Verhamme C, Eftimov F, et al. Prognostic value of nerve ultrasonography: A prospective multicenter study on the natural history of chronic inflammatory neuropathies. Eur J Neurol 2021;28(7):2327-2338.doi:10.1111/ene.14885
  44. Goedee HS, van der Pol WL, van Asseldonk JH, Franssen H, Notermans NC, Vrancken AJ, et al. Diagnostic value of sonography in treatment-naive chronic inflammatory neuropathies. Neurology 2017;88(2):143-151.doi:10.1212/WNL.0000000000003483
  45. Fisse AL, Pitarokoili K, Motte J, Gamber D, Kerasnoudis A, Gold R, et al. Nerve echogenicity and intranerve CSA variability in high-resolution nerve ultrasound (HRUS) in chronic inflammatory demyelinating polyneuropathy (CIDP). J Neurol 2019;266(2):468-475.doi:10.1007/s00415-018-9158-3
  46. Delmont E, Azulay JP, Giorgi R, Attarian S, Verschueren A, Uzenot D, et al. Multifocal motor neuropathy with and without conduction block: a single entity? Neurology 2006;67(4):592-596.doi:10.1212/01.wnl.0000234063.51897.20
  47. van Rosmalen MHJ, Goedee HS, van der Gijp A, Witkamp TD, van Eijk RPA, Asselman FL, et al. Quantitative assessment of brachial plexus MRI for the diagnosis of chronic inflammatory neuropathies. J Neurol 2021;268(3):978-988.doi:10.1007/s00415-020-10232-8
  48. Oudeman J, Eftimov F, Strijkers GJ, Schneiders JJ, Roosendaal SD, Engbersen MP, et al. Diagnostic accuracy of MRI and ultrasound in chronic immune-mediated neuropathies. Neurology 2020;94(1):e62-e74.doi:10.1212/wnl.0000000000008697
  49. Goedee HS, Herraets IJT, Visser LH, Franssen H, van Asseldonk JH, van der Pol WL, et al. Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination. Muscle Nerve 2019;60(4):415-419.doi:10.1002/mus.26629
  50. Beekman R, van den Berg LH, Franssen H, Visser LH, van Asseldonk JT, Wokke JH. Ultrasonography shows extensive nerve enlargements in multifocal motor neuropathy. Neurology 2005;65(2):305-307.doi:10.1212/01.wnl.0000169179.67764.30
  51. Kerasnoudis A, Pitarokoili K, Behrendt V, Gold R, Yoon MS. Multifocal motor neuropathy: correlation of nerve ultrasound, electrophysiological, and clinical findings. J Peripher Nerv Syst 2014;19(2):165-174.doi:10.1111/jns5.12067
  52. Rattay TW, Winter N, Decard BF, Dammeier NM, Hartig F, Ceanga M, et al. Nerve ultrasound as follow-up tool in treated multifocal motor neuropathy. Eur J Neurol 2017;24(9):1125-1134.doi:10.1111/ene.13344
  53. Pitarokoili K, Gold R, Yoon MS. Nerve ultrasound in a case of multifocal motor neuropathy without conduction block. Muscle Nerve 2015;52(2):294-299.doi:10.1002/mus.24583
  54. Li Y, Niu J, Liu T, Ding Q, Wu S, Guan Y, et al. Conduction Block and Nerve Cross-Sectional Area in Multifocal Motor Neuropathy. Front Neurol 2019;10(1055.doi:10.3389/fneur.2019.01055
  55. Padua L, Martinoli C, Pazzaglia C, Lucchetta M, Granata G, Erra C, et al. Intra- and internerve cross-sectional area variability: new ultrasound measures. Muscle Nerve 2012;45(5):730-733.doi:10.1002/mus.23252
  56. Grimm A, Décard BF, Athanasopoulou I, Schweikert K, Sinnreich M, Axer H. Nerve ultrasound for differentiation between amyotrophic lateral sclerosis and multifocal motor neuropathy. J Neurol 2015;262(4):870-880.doi:10.1007/s00415-015-7648-0
  57. Loewenbrück KF, Liesenberg J, Dittrich M, Schäfer J, Patzner B, Trausch B, et al. Nerve ultrasound in the differentiation of multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis with predominant lower motor neuron disease (ALS/LMND). J Neurol 2016;263(1):35-44.doi:10.1007/s00415-015-7927-9
  58. Tsuji Y, Noto YI, Kitaoji T, Kojima Y, Mizuno T. Difference in distribution of fasciculations between multifocal motor neuropathy and amyotrophic lateral sclerosis. Clin Neurophysiol 2020;131(12):2804-2808.doi:10.1016/j.clinph.2020.08.021
  59. Liu J, Li Y, Niu J, Zhang L, Fan J, Guan Y, et al. Fasciculation differences between ALS and non-ALS patients: an ultrasound study. BMC Neurol 2021;21(1):441.doi:10.1186/s12883-021-02473-5
  60. Grimm A, Décard BF, Bischof A, Axer H. Ultrasound of the peripheral nerves in systemic vasculitic neuropathies. J Neurol Sci 2014;347(1-2):44-49.doi:10.1016/j.jns.2014.09.017
  61. Goedee HS, van der Pol WL, van Asseldonk JH, Vrancken A, Notermans NC, Visser LH, et al. Nerve sonography to detect peripheral nerve involvement in vasculitis syndromes. Neurol Clin Pract 2016;6(4):293-303.doi:10.1212/cpj.0000000000000258
  62. Ito T, Kijima M, Watanabe T, Sakuta M, Nishiyama K. Ultrasonography of the tibial nerve in vasculitic neuropathy. Muscle Nerve 2007;35(3):379-382.doi:10.1002/mus.20673
  63. Nodera H, Sato K, Terasawa Y, Takamatsu N, Kaji R. High-resolution sonography detects inflammatory changes in vasculitic neuropathy. Muscle Nerve 2006;34(3):380-381.doi:10.1002/mus.20582
  64. Razali SNO, Arumugam T, Yuki N, Rozalli FI, Goh KJ, Shahrizaila N. Serial peripheral nerve ultrasound in Guillain-Barré syndrome. Clin Neurophysiol 2016;127(2):1652-1656.doi:10.1016/j.clinph.2015.06.030
  65. Grimm A, Décard BF, Schramm A, Pröbstel AK, Rasenack M, Axer H, et al. Ultrasound and electrophysiologic findings in patients with Guillain-Barré syndrome at disease onset and over a period of six months. Clin Neurophysiol 2016;127(2):1657-1663.doi:10.1016/j.clinph.2015.06.032
  66. Almeida V, Mariotti P, Veltri S, Erra C, Padua L. Nerve ultrasound follow-up in a child with Guillain-Barré syndrome. Muscle Nerve 2012;46(2):270-275.doi:10.1002/mus.23325
  67. Berciano J, Orizaola P, Gallardo E, Pelayo-Negro AL, Sánchez-Juan P, Infante J, et al. Very early Guillain-Barré syndrome: A clinical-electrophysiological and ultrasonographic study. Clin Neurophysiol Pract 2020;5(1-9.doi:10.1016/j.cnp.2019.11.003
  68. Gallardo E, Sedano MJ, Orizaola P, Sánchez-Juan P, González-Suárez A, García A, et al. Spinal nerve involvement in early Guillain-Barré syndrome: a clinico-electrophysiological, ultrasonographic and pathological study. Clin Neurophysiol 2015;126(4):810-819.doi:10.1016/j.clinph.2014.06.051
  69. Grimm A, Décard BF, Axer H. Ultrasonography of the peripheral nervous system in the early stage of Guillain-Barré syndrome. J Peripher Nerv Syst 2014;19(3):234-241.doi:10.1111/jns.12091
  70. Berciano J, Gallardo E, Orizaola P, de Lucas EM, García A, Pelayo-Negro AL, et al. Early axonal Guillain-Barré syndrome with normal peripheral conduction: imaging evidence for changes in proximal nerve segments. J Neurol Neurosurg Psychiatry 2016;87(5):563-565.doi:10.1136/jnnp-2015-310601
  71. Grimm A, Décard BF, Axer H, Fuhr P. The Ultrasound pattern sum score - UPSS. A new method to differentiate acute and subacute neuropathies using ultrasound of the peripheral nerves. Clin Neurophysiol 2015;126(11):2216-2225.doi:10.1016/j.clinph.2015.01.011
  72. Mori A, Nodera H, Takamatsu N, Maruyama-Saladini K, Osaki Y, Shimatani Y, et al. Sonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome. J Neurol Sci 2016;364(154-159.doi:10.1016/j.jns.2016.03.042
  73. Liu L, Ye Y, Wang L, Song X, Cao J, Qi Y, et al. Nerve ultrasound evaluation of Guillain-Barré syndrome subtypes in northern China. Muscle Nerve 2021;64(5):560-566.doi:10.1002/mus.27386
  74. Decard BF, Fladt J, Axer H, Fischer D, Grimm A. Nerve ultrasound in miller fisher variant of Guillain-Barre syndrome. Muscle Nerve 2015.doi:10.1002/mus.24753

How to Cite

“Progress in the Nerve Ultrasound of Inflammatory Peripheral Neuropathies”. Human Brain, vol. 2, no. 3, Jan. 2024, https://doi.org/10.37819/hb.3.1776.

How to Cite

“Progress in the Nerve Ultrasound of Inflammatory Peripheral Neuropathies”. Human Brain, vol. 2, no. 3, Jan. 2024, https://doi.org/10.37819/hb.3.1776.

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