Clinical heterogeneity of chronic inflammatory demyelinating polyneuropathy: diagnostic challenges

Cover Page

Cite item


Сhronic inflammatory demyelinating polyneuropathy (CIDP) is the most prevalent acquired dysimmune neuropathy with clinical picture of symmetric motor and sensory disturbances. Since the first description of CIDP, many atypical variants have been described, which may reach up to 50% of cases. Diagnosis of atypical CIDP may be challenging due to different clinical presentation and treatment response. Current researches improve our knowledge about dysimmune neuropathies and highlight the importance of its classification. Nowadays CIDP is considered as a spectrum of disorders rather than a separate disease entity. Up to date, more than 15 diagnostic criteria have been proposed reflecting the complexity of СIDP diagnosis. Many polyneuropathies may mimic CIDP, therefore CIDP is frequently a diagnosis of exclusion. The key diagnostic instrument is electroneuromyography; however, the issues related to results misinterpretation and some technical aspects are the most important in CIDP misdiagnosis. Supportive instrumental and laboratory methods have variable sensitivity and specificity, making challenging CIDP diagnosis, especially its atypical forms. The importance of an early and accurate diagnosis of CIDP is supported by an effective pathogenic treatment, which affects the patient's prognosis and level of disability.

About the authors

A. S. Rizvanova

Research Center of Neurology

Author for correspondence.
ORCID iD: 0000-0002-9890-3552

Alina S. Rizvanova – MD, Neurologist, Center of Peripheral Nerve Disorders.

80 Volokolamskoe shosse, Moscow, 125367
Tel.: +7 (967) 250 37 93 

Russian Federation

D. A. Grishina

Research Center of Neurology

ORCID iD: 0000-0002-7924-3405

Daria A. Grishina – MD, PhD, Head of the Center of Peripheral Nerve Disorders.

80 Volokolamskoe shosse, Moscow, 125367

Russian Federation

N. A. Suponeva

Research Center of Neurology

ORCID iD: 0000-0003-3956-6362

Natalia A. Suponeva – MD, PhD, Professor, Corr. Member of Russ. Acad. Sci., Head of the Department of Neurorehabilitation and Physiotherapy.

80 Volokolamskoe shosse, Moscow, 125367

Russian Federation


  1. Lozeron P, Mariani LL, Dodet P, Beaudonnet G, Théaudin M, Adam C, Arnulf B, Adams D. Transthyretin amyloid polyneuropathies mimicking a demyelinating polyneuropathy. Neurology. 2018;91(2):e143–52. doi: 10.1212/WNL.0000000000005777.
  2. Dyck PJB, Tracy JA. History, Diagnosis, and Management of Chronic Inflammatory Demyelinating Polyradiculoneuropathy. Mayo Clin Proc. 2018;93(6):777–93. doi: 10.1016/j.mayocp.2018.03.026.
  3. Broers MC, Bunschoten C, Nieboer D, Lingsma HF, Jacobs BC. Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis. Neuroepidemiology. 2019;52(3–4):161–72. doi: 10.1159/000494291.
  4. Allen JA, Lewis RA. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology. 2015;85:498–504.
  5. Allen JA, Gorson KC, Gelinas D. Challenges in the diagnosis of chronic inflammatory demyelinating polyneuropathy. Brain Behav. 2018;8(3):e00932. doi: 10.1002/brb3.932.
  6. Neligan A, Reilly MM, Lunn MP. CIDP: mimics and chameleons. Pract Neurol. 2014;14(6):399–408. doi: 10.1136/practneurol-2014-000831.
  7. Lehmann HC, Burke D, Kuwabara S. Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry. 019;90(9):981–7. doi: 10.1136/jnnp-2019-320314.
  8. Doneddu PE, Cocito D, Manganelli F, Fazio R, Briani C, Filosto M, Benedetti L, Mazzeo A, Marfia GA, Cortese A, Fierro B, Jann S, Beghi E, Clerici AM, Carpo M, Schenone A, Luigetti M, Lauria G, Antonini G, Rosso T, Siciliano G, Cavaletti G, Liberatore G, Santoro L, Peci E, Tronci S, Ruiz M, Cotti Piccinelli S, Toscano A, Mataluni G, Piccolo L, Cosentino G, Sabatelli M, Nobile-Orazio E; Italian CIDP Database study group. Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry. 2019;90(2):125–32. doi: 10.1136/jnnp-2018-318714.
  9. Devaux JJ, Miura Y, Fukami Y, Inoue T, Manso C, Belghazi M, Sekiguchi K, Kokubun N, Ichikawa H, Wong AH, Yuki N. Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy. Neurology. 2016;86(9):800–7. doi: 10.1212/WNL.0000000000002418.
  10. Delmont E, Manso C, Querol L, Cortese A, Berardinelli A, Lozza A, Belghazi M, Malissart P, Labauge P, Taieb G, Yuki N, Illa I, Attarian S, Devaux JJ. Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy. Brain. 2017;140(7): 1851–8. doi: 10.1093/brain/awx124.
  11. Kuwabara S, Isose S, Mori M, Mitsuma S, Sawai S, Beppu M, Sekiguchi Y, Misawa S. Different electrophysiological profiles and treatment response in 'typical' and 'atypical' chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2015;86(10):1054–9. doi: 10.1136/jnnp-2014-308452.
  12. Mahdi-Rogers M, Hughes RA. Epidemiology of chronic inflammatory neuropathies in Southeast England. Eur J Neurol. 2014;21(1):28–33. doi: 10.1111/ene.12190.
  13. Bunschoten C, Jacobs BC, Van den Bergh PYK, Cornblath DR, van Doorn PA. Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol. 2019;18(8):784–94. doi: 10.1016/S1474-4422(19)30144-9.
  14. Kuwabara S, Misawa S. Chronic Inflammatory Demyelinating Polyneuropathy. Adv Exp Med Biol. 2019;1190:333–43. doi: 10.1007/978-981-32-9636-7_21.
  15. Супонева НА, Наумова ЕС, Гнедовская ЕВ. Хроническая воспалительная демиелинизирующая полинейропатия у взрослых: принципы диагностики и терапия первой линии. Нервно-мышечные болезни. 2016;6(1):44–53. doi: 10.17650/2222-8721-2016-6-1-44-53.
  16. London ZN, Nowacek DG. Does cerebrospinal fluid analysis have a meaningful role in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy? Muscle Nerve. 2019;60(2):111–3. doi: 10.1002/mus.26513.
  17. Breiner A, Moher D, Brooks J, Cheng W, Hegen H, Deisenhammer F, McCudden CR, Bourque PR. Adult CSF total protein upper reference limits should be age-partitioned and significantly higher than 0.45 g/L: a systematic review. J Neurol. 2019;266(3):616–24. doi: 10.1007/s00415-018-09174-z.
  18. Jomier F, Bousson V, Viala K, Péréon Y, Magot A, Cauquil C, Bouhour F, Vial C, Bedat-Millet AL, Taithe F, Bresch S, Siri A, Kubis N, Lozeron P. Prospective study of the additional benefit of plexus magnetic resonance imaging in the diagnosis of chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2020;27(1):181–7. doi: 10.1111/ene.14053.
  19. Lozeron P, Lacour MC, Vandendries C, Théaudin M, Cauquil C, Denier C, Lacroix C, Adams D. Contribution of plexus MRI in the diagnosis of atypical chronic inflammatory demyelinating polyneuropathies. J Neurol Sci. 2016;360:170–5. doi: 10.1016/j.jns.2015.11.048.
  20. Василенко АФ, Шестакова МВ, Кочетков ИВ, Карпова МИ. Магнитно-резонансная томография конского хвоста при хронической воспалительной демиелинизирующей полинейропатии. Нервно-мышечные болезни. 2017;7(4):44–9. doi: 10.17650/2222-8721-2017-7-4-44-49.
  21. Дружинин ДС, Наумова ЕС, Никитин СС. Ультразвуковая визуализация периферических нервов при мультифокальной моторной нейропатии и хронической воспалительной демиелинизирующей полинейропатии. Нервно-мышечные болезни. 2016;6(1): 63–73. doi: 10.17650/2222-8721-2016-6-1-63-73.
  22. 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): 2216–25. doi: 10.1016/j.clinph.2015.01.011.
  23. Kerasnoudis A, Pitarokoili K, Behrendt V, Gold R, Yoon MS. Bochum ultrasound score versus clinical and electrophysiological parameters in distinguishing acute-onset chronic from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2015;51(6):846–52. doi: 10.1002/mus.24484.
  24. Padua L, Granata G, Sabatelli M, Inghilleri M, Lucchetta M, Luigetti M, Coraci D, Martinoli C, Briani C. Heterogeneity of root and nerve ultrasound pattern in CIDP patients. Clin Neurophysiol. 2014;125(1):160–5. doi: 10.1016/j.clinph.2013.07.023.
  25. 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):635–41. doi: 10.1016/j.clinph.2013.08.014.
  26. Kerasnoudis A, Pitarokoili K, Haghikia A, Gold R, Yoon MS. Nerve ultrasound protocol in differentiating chronic immune-mediated neuropathies. Muscle Nerve. 2016;54(5):864–71. doi: 10.1002/mus.25138.
  27. Stettner M, Hinrichs L, Guthoff R, Bairov S, Petropoulos IN, Warnke C, Hartung HP, Malik RA, Kieseier BC. Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy. Ann Clin Transl Neurol. 2015;3(2): 88–100. doi: 10.1002/acn3.275.
  28. Querol L, Devaux J, Rojas-Garcia R, Illa I. Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications. Nat Rev Neurol. 2017;13(9):533–47. doi: 10.1038/nrneurol.2017.84.
  29. Kieseier BC, Mathey EK, Sommer C, Hartung HP. Immune-mediated neuropathies. Nat Rev Dis Primers. 2018;4(1):31. doi: 10.1038/s41572-018-0027-2.
  30. Wanleenuwat P, Iwanowski P, Kozubski W. Antiganglioside antibodies in neurological diseases. J Neurol Sci. 2020;408:116576. doi: 10.1016/j.jns.2019.116576.
  31. Kaida K. Guillain-Barré Syndrome. Adv Exp Med Biol. 2019;1190:323–31. doi: 10.1007/978-981-32-9636-7_20.
  32. Esposito S, Longo MR. Guillain-Barré syndrome. Autoimmun Rev. 2017;16(1):96–101. doi: 10.1016/j.autrev.2016.09.022.
  33. Beadon K, Guimarães-Costa R, Léger JM. Multifocal motor neuropathy. Curr Opin Neurol. 2018;31(5):559–64. doi: 10.1097/WCO.0000000000000605.
  34. 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):140–8. doi: 10.1136/jnnp-2019-321532.
  35. Lunn MP. Neuropathies and paraproteins. Curr Opin Neurol. 2019;32(5):658–65. doi: 10.1097/WCO.0000000000000726.
  36. Rison RA, Beydoun SR. Paraproteinemic neuropathy: a practical review. BMC Neurol. 2016;16:13. doi: 10.1186/s12883-016-0532-4.
  37. Rajabally YA, Stettner M, Kieseier BC, Hartung HP, Malik RA. CIDP and other inflammatory neuropathies in diabetes – diagnosis and management. Nat Rev Neurol. 2017;13(10): 599–611. doi: 10.1038/nrneurol.2017.123.
  38. Bril V, Blanchette CM, Noone JM, Runken MC, Gelinas D, Russell JW. The dilemma of diabetes in chronic inflammatory demyelinating polyneuropathy. J Diabetes Complications. 2016;30(7):1401–7. doi: 10.1016/j.jdiacomp.2016.05.007.
  39. Özel G, Maisonobe T, Guyant-Maréchal L, Maltête D, Lefaucheur R. Hereditary neuropathy with liability to pressure palsies mimicking chronic inflammatory demyelinating poly-neuropathy. Rev Neurol (Paris). 2018;174(7–8): 575–7. doi: 10.1016/j.neurol.2017.11.012.

Supplementary files

There are no supplementary files to display.

Copyright (c) 2020 Rizvanova A.S., Grishina D.A., Suponeva N.A.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies