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DOI: 10.1055/a-2731-5029
Nerve Ultrasound in Pediatric Polyneuropathies: A Systematic Review
Authors
Funding Information None.
Abstract
The diagnosis of peripheral polyneuropathy in children and the differential diagnosis among its various forms often present a challenge, also because electrodiagnostic studies can be painful and sometimes yield inconclusive results. This systematic review examines the role of nerve ultrasound (n-US) in the diagnosis and follow-up of pediatric polyneuropathies. We searched PubMed and Embase from 1975 to April 1, 2025. Included studies assessed patients aged ≤ 18 years with clinically and neurophysiologically confirmed polyneuropathy, providing pediatric-specific qualitative or quantitative n-US findings. Eighteen studies met the inclusion criteria. Six focused on acquired inflammatory polyneuropathies (three on Guillain–Barré Syndrome [GBS], three on Chronic Inflammatory Demyelinating Polyneuropathy [CIDP]), eight on Charcot–Marie–Tooth disease (CMT), two on lysosomal storage disorders, one on Autosomal Recessive Spastic Ataxia of Charlevoix–Saguenay (ARSACS), and one on mixed etiologies. Most (n = 7) were case reports. Cross-sectional area and nerve enlargement (NE) distribution were the main parameters evaluated. Marked, diffuse NE was found in demyelinating CMT and lysosomal disorders; CIDP showed diffuse and multifocal NE; GBS presented mild and proximal NE. No NE was reported in axonal CMT or ARSACS. Few studies assessed echogenicity or fascicular structure; none evaluated vascularization. n-US shows promise in differentiating demyelinating conditions such as CMT, CIDP, GBS, and certain metabolic syndromes in children. However, further age-matched control studies are needed, given that nerve growth and myelination peak between 15 and 17 years. Future research should explore n-US as an early diagnostic, screening, and follow-up tool.
Keywords
systematic review - children acquired - inherited polyneuropathy - nerve ultrasound - cross-sectional area - nerve enlargementContributors' Statement
All authors contributed to the study's conception and design, commented on previous versions of the manuscript, and read and approved the final version. Material preparation, data collection, and analysis were performed by S.M. and A.P., who also wrote the first draft of the manuscript.
Ethical Approval
Not applicable.
Publication History
Received: 28 July 2025
Accepted: 21 October 2025
Accepted Manuscript online:
27 October 2025
Article published online:
03 November 2025
© 2025. Thieme. All rights reserved.
Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
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References
- 1 Ouvrier R. Peripheral neuropathies in the young child. Rev Neurol (Paris) 2004; 160 (12) 1216-1220
- 2 Paketci C, Karakaya M, Edem P. et al. Clinical, electrophysiological and genetic characteristics of childhood hereditary polyneuropathies. Rev Neurol (Paris) 2020; 176 (10) 846-855
- 3 Alshaikh NM, Martinez JP, Pitt MC. Perception of pain during electromyography in children: a prospective study. Muscle Nerve 2016; 54 (03) 422-426
- 4 McMillan HJ, Ryan MM. Overview of pediatric peripheral neuropathies. Neuromuscular Disorders of Infancy, Childhood, and Adolescence 2015: 274-288
- 5 van Doorn PA, Van den Bergh PYK, Hadden RDM. et al. European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome. J Peripher Nerv Syst 2023; 28 (04) 535-563
- 6 Morena J, Gupta A, Hoyle JC. Charcot-Marie-Tooth: from molecules to therapy. Int J Mol Sci 2019; 20 (14) 3419
- 7 Pisciotta C, Shy ME. Hereditary neuropathy. Handb Clin Neurol 2023; 195: 609-617
- 8 Braathen GJ. Genetic epidemiology of Charcot-Marie-Tooth disease. Acta Neurol Scand Suppl 2012; 193 (193) iv-22
- 9 Saporta MA, Shy ME. Inherited peripheral neuropathies. Neurol Clin 2013; 31 (02) 597-619
- 10 Stojkovic T. Hereditary neuropathies: AN update. Rev Neurol (Paris) 2016; 172 (12) 775-778
- 11 Plante-Bordeneuve V, Said G. Dejerine-Sottas disease and hereditary demyelinating polyneuropathy of infancy. Muscle Nerve 2002; 26 (05) 608-621
- 12 Higuchi Y, Takashima H. Clinical genetics of Charcot-Marie-Tooth disease. J Hum Genet 2023; 68 (03) 199-214
- 13 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) 3556-3583 Erratum in: Eur J Neurol 29(4):1288
- 14 Fernandez-Garcia MA, Stettner GM, Kinali M. et al. Genetic neuropathies presenting with CIDP-like features in childhood. Neuromuscul Disord 2021; 31 (02) 113-122
- 15 Łukawska M, Potulska-Chromik A, Lipowska M. et al. Pediatric CIDP: Diagnosis and Management. A Single-Center Experience. Front Neurol 2021; 12: 667378
- 16 Puma A, Azulay N, Grecu N. et al. Comparison of high-frequency and ultrahigh-frequency probes in chronic inflammatory demyelinating polyneuropathy. J Neurol 2019; 266 (09) 2277-2285
- 17 Telleman JA, Grimm A, Goedee S, Visser LH, Zaidman CM. Nerve ultrasound in polyneuropathies. Muscle Nerve 2018; 57 (05) 716-728
- 18 Tagliafico A, Cadoni A, Fisci E, Bignotti B, Padua L, Martinoli C. Reliability of side-to-side ultrasound cross-sectional area measurements of lower extremity nerves in healthy subjects. Muscle Nerve 2012; 46 (05) 717-722
- 19 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 (03) 457-462
- 20 Draaisma F, Leenders EKSM, Erasmus CE. et al. Nerve enlargement in patients with Noonan syndrome: a retrospective cohort study. Am J Med Genet A 2024; 194 (11) e63810
- 21 Voltan G, Marques-Júnior W, Santana JM. et al. Silent peripheral neuropathy determined by high-resolution ultrasound among contacts of patients with Hansen's disease. Front Med (Lausanne) 2023; 9: 1059448
- 22 Telleman JA, Stellingwerff MD, Brekelmans GJ, Visser LH. Nerve ultrasound: a useful screening tool for peripheral nerve sheath tumors in NF1?. Neurology 2017; 88 (17) 1615-1622
- 23 Grimm AS, Schubert C, Grimm A. et al. Normative observational nerve ultrasound values in school-age children and adolescents and their application to hereditary neuropathies. Front Neurol 2020; 11: 303
- 24 Cartwright MS, Shin HW, Passmore LV, Walker FO. Ultrasonographic reference values for assessing the normal median nerve in adults. J Neuroimaging 2009; 19 (01) 47-51
- 25 Cartwright MS, Mayans DR, Gillson NA, Griffin LP, Walker FO. Nerve cross-sectional area in extremes of age. Muscle Nerve 2013; 47 (06) 890-893
- 26 Schubert C, Grimm AS, Stahl JH. et al. Nerve ultrasound reference data in children from two to seven years. Clin Neurophysiol 2020; 131 (04) 859-865
- 27 Page MJ, McKenzie JE, Bossuyt PM. et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. BMJ 2021; 372 (71) n71
- 28 Gorson KC, Ropper AH, Muriello MA, Blair R. Prospective evaluation of MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome. Neurology 1996; 47 (03) 813-817
- 29 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 (03) 234-241
- 30 Zaidman CM, Harms MB, Pestronk A. Ultrasound of inherited vs. acquired demyelinating polyneuropathies. J Neurol 2013; 260 (12) 3115-3121
- 31 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
- 32 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 (02) 270-275
- 33 Prado Jr MB, Narito KM, Adiao KJB. Anti-GM1 IgM antibody positive axonal variant of Guillain-Barre-syndrome in a pediatric patient with dengue fever. J Neuroimmunol 2021; 355: 577572
- 34 Décard BF, Fladt J, Axer H, Fischer D, Grimm A. Nerve ultrasound in Miller Fisher variant of Guillain-Barré syndrome. Muscle Nerve 2015; 52 (06) 1106-1110
- 35 Padua L, Granata G, Sabatelli M. et al. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol 2014; 125 (01) 160-165
- 36 Goedee HS, Brekelmans GJ, Visser LH. Multifocal enlargement and increased vascularization of peripheral nerves detected by sonography in CIDP: a pilot study. Clin Neurophysiol 2014; 125 (01) 154-159
- 37 Puma A, Grecu N, Badea RȘ. et al. Typical CIDP, distal variant CIDP, and anti-MAG antibody neuropathy: An ultra-high frequency ultrasound comparison of nerve structure. Sci Rep 2024; 14 (01) 4643
- 38 Grimm A, Vittore D, Schubert V. et al. Ultrasound aspects in therapy-naive CIDP compared to long-term treated CIDP. J Neurol 2016; 263 (06) 1074-1082
- 39 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 (08) 803-809
- 40 Kerasnoudis A, Pitarokoili K, Gold R, Yoon MS. Nerve ultrasound and electrophysiology for therapy monitoring in chronic inflammatory demyelinating polyneuropathy. J Neuroimaging 2015; 25 (06) 931-939
- 41 Härtig F, Ross M, Dammeier NM. et al. Nerve ultrasound predicts treatment response in chronic inflammatory demyelinating polyradiculoneuropathy-a prospective follow-up. Neurotherapeutics 2018; 15 (02) 439-451
- 42 Lucchetta M, Vidal E, Sartori S. et al. Long-term plasma exchange in pediatric CIDP. J Clin Apher 2015; 30 (06) 364-366
- 43 VanHorn TA, Cartwright MS. Neuromuscular ultrasound in the pediatric population. Diagnostics (Basel) 2020; 10 (12) 1012
- 44 Zanette G, Fabrizi GM, Taioli F. et al. Nerve ultrasound findings differentiate Charcot-Marie-Tooth disease (CMT) 1A from other demyelinating CMTs. Clin Neurophysiol 2018; 129 (11) 2259-2267
- 45 Goedee SH, Brekelmans GJ, van den Berg LH, Visser LH. Distinctive patterns of sonographic nerve enlargement in Charcot-Marie-Tooth type 1A and hereditary neuropathy with pressure palsies. Clin Neurophysiol 2015; 126 (07) 1413-1420
- 46 Padua L, Coraci D, Lucchetta M. et al. Different nerve ultrasound patterns in charcot-marie-tooth types and hereditary neuropathy with liability to pressure palsies. Muscle Nerve 2018; 57 (01) E18-E23
- 47 Noto Y, Shiga K, Tsuji Y. et al. Nerve ultrasound depicts peripheral nerve enlargement in patients with genetically distinct Charcot-Marie-Tooth disease. J Neurol Neurosurg Psychiatry 2015; 86 (04) 378-384
- 48 Yiu EM, Brockley CR, Lee KJ. et al. Peripheral nerve ultrasound in pediatric Charcot-Marie-Tooth disease type 1A. Neurology 2015; 84 (06) 569-574
- 49 Schreiber S, Oldag A, Kornblum C. et al. Sonography of the median nerve in CMT1A, CMT2A, CMTX, and HNPP. Muscle Nerve 2013; 47 (03) 385-395
- 50 Hobbelink SMR, Brockley CR, Kennedy RA. et al. Dejerine-Sottas disease in childhood-Genetic and sonographic heterogeneity. Brain Behav 2018; 8 (04) e00919
- 51 Yiu EM, Wanigasinghe J, Mackay MT, Gonzales M, Nicholson GA, Ryan MM. Infantile-onset myelin protein zero-related demyelinating neuropathy presenting as an upper extremity monoplegia. Semin Pediatr Neurol 2018; 26: 52-55
- 52 Wu R, Fu J, Meng L. et al. Homozygous splice-site mutation c.78 + 5G>A in PMP22 causes congenital hypomyelinating neuropathy. Neuropathology 2019; 39 (06) 441-446
- 53 Castoro R, Crisp J, Caress JB, Li J, Cartwright MS. Segmental nerve enlargement in CMT4J. Muscle Nerve 2020; 61 (06) E44-E46
- 54 Bayrak AO, Bayrak IK, Battaloglu E, Ozes B, Yildiz O, Onar MK. Ultrasonographic findings in hereditary neuropathy with liability to pressure palsies. Neurol Res 2015; 37 (02) 106-111
- 55 Ginanneschi F, Filippou G, Giannini F. et al. Sonographic and electrodiagnostic features of hereditary neuropathy with liability to pressure palsies. J Peripher Nerv Syst 2012; 17 (04) 391-398
- 56 Grimm A, Schäffer E, Just J. et al. Thickening of the peripheral nerves in metachromatic leukodystrophy. J Neurol Sci 2016; 368: 399-401
- 57 Küpper H, Kaiser N, Winter N. et al. Enlargement of peripheral nerves in Krabbe disease: the diagnostic value of nerve ultrasound. Muscle Nerve 2020; 61 (04) E24-E27
- 58 Kneer K, Straub S, Wittlinger J. et al. Neuropathy in ARSACS is demyelinating but without typical nerve enlargement in nerve ultrasound. J Neurol 2024; 271 (05) 2494-2502
- 59 Behse F. Morphometric studies on the human sural nerve. Acta Neurol Scand Suppl 1990; 132 (suppl): 1-38
- 60 Gutrecht JA, Dyck PJ. Quantitative teased-fiber and histologic studies of human sural nerve during postnatal development. J Comp Neurol 1970; 138 (01) 117-129
- 61 Zaidman CM, Al-Lozi M, Pestronk A. Peripheral nerve size in normals and patients with polyneuropathy: an ultrasound study. Muscle Nerve 2009; 40 (06) 960-966
- 62 Lothet EH, Bishop TJ, Walker FO, Cartwright MS. Ultrasound-derived nerve cross-sectional area in extremes of height and weight. J Neuroimaging 2019; 29 (03) 406-409
- 63 Gabreëls-Festen AA, Joosten EM, Gabreëls FJ, Jennekens FG, Janssen-van Kempen TW. Early morphological features in dominantly inherited demyelinating motor and sensory neuropathy (HMSN type I). J Neurol Sci 1992; 107 (02) 145-154
- 64 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 (04) 2425-2435
- 65 Palu E, Järvilehto J, Pennonen J. et al. Rare PMP22 variants in mild to severe neuropathy uncorrelated to plasma GDF15 or neurofilament light. Neurogenetics 2023; 24 (04) 291-301
- 66 Haw TT, Foo LK, Geap TC. et al. P-PN023. Childhood-onset demyelinating polyneuropathy: challenges in differentiating acquired from genetic disease. Clin Neurophysiol 2021; 132 (08) e113
- 67 Hobson-Webb LD, Massey JM, Juel VC. Nerve ultrasound in diabetic polyneuropathy: correlation with clinical characteristics and electrodiagnostic testing. Muscle Nerve 2013; 47 (03) 379-384
- 68 Arumugam T, Razali SN, Vethakkan SR, Rozalli FI, Shahrizaila N. Relationship between ultrasonographic nerve morphology and severity of diabetic sensorimotor polyneuropathy. Eur J Neurol 2016; 23 (02) 354-360
- 69 Riazi S, Bril V, Perkins BA. et al. Can ultrasound of the tibial nerve detect diabetic peripheral neuropathy? A cross-sectional study. Diabetes Care 2012; 35 (12) 2575-2579
- 70 Heiling B, Kneer K, He W. et al. Nerve ultrasound helps to distinguish CIDP patients with diabetes from patients with diabetic polyneuropathy. Sci Rep 2024; 14 (01) 30504
- 71 Eilers MC, Fellmann M, l'Allemand D. et al. Small fibre neuropathy detected by ultrasound of the median nerve in children with type 1 diabetes mellitus is related to glycemic control [preprint]. medRxiv. 2025
- 72 Tsai WC, Chiou HJ, Chou YH, Wang HK, Chiou SY, Chang CY. Differentiation between schwannomas and neurofibromas in the extremities and superficial body: the role of high-resolution and color Doppler ultrasonography. J Ultrasound Med 2008; 27 (02) 161-166 , quiz 168–169
- 73 Rafailidis V, Kaziani T, Theocharides C, Papanikolaou A, Rafailidis D. Imaging of the malignant peripheral nerve sheath tumour with emphasis οn ultrasonography: correlation with MRI. J Ultrasound 2014; 17 (03) 219-223
