Chronic inflammatory demyelinating polyradiculoneuropathy

Česká verzia

Authors: E. Ehler
Authors place of work: Neurologická klinika FZS UP a Pardubické krajské nemocnice, a. s.
Published in the journal: Cesk Slov Neurol N 2018; 81(3): 252-265
Category: Minimonography
doi: https://doi.org/10.14735/amcsnn2018252

Summary

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic demyelinating polyneuropathy with a relapsing or progressive course. This is a dysimmune polyneuropathy with activation of humoral and cellular mechanisms with a primary lesion of myelin sheath in the peripheral nerves. Most frequent is a typical symmetrical polyneuropathy, multifocal neuropathy with a conduction block is less frequent, then distal symmetrical motor and sensory neuropathy, pure sensory or pure motor neuropathies occur rarely. Development of signs and symptoms beyond 2 months is a common feature for all variants. Diagnosis of CIDP is based on clinical findings, precisely developed electrophysiological criteria, and some laboratory findings – hyperproteinorrhachia with normal cell count, and MRI with thickening and enhancement of nerves in brachalis and lumbar plexuses or roots. Corticosteroids or intravenous immunoglobulins are used as induction therapy as the first-line treatment of CIDP. Therapeutic plasma exchange is a second-line therapy in patients with inefficacy or intolerability of the first-line treatment. As adjuvant therapy, they use azathioprin, mycofenolate mofetil, ciclosporin A, cyclophosphamide, and methotrexate. CIDP is a treatable neuropathy though in some patients it leads to invalidity.

Key words:
chronic inflammatory demyelinating neuropathy – demyelination and remyelination – electrophysiology – diagnostics – immunotherapy.

The author declares he has no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Zdroje

1. Kuwabara S, Isose S, Mori M et al. Different electrophysiological profiles in „typical“ and „atypical“ chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2015; 86(10): 1054–1059. doi: 10.1136/jnnp-2014-308452.

2. Bednařík J. Chronická zánětlivá demyelinizační neuropatie. Neurol praxi 2016; 17(1): 16–21.

3. Overell S, Wilson HJ. Chronic inflammatory polyradiculoneuropathy: classification and treatment. Practical Neurology 2006; 6: 102–110.

4. Pourmand R. Immune-mediated neuromuscular diseases. Basel: Karger 2009.

5. Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic chalnges for a treatable condition. Lancet Neurol 2010; 9(4): 402–412. doi: 10.1016/S1474-4422(10)70041-7.

6. Jo HY, Park MG, Kim DS et al. Chronic inflammatory demyelinating polyradiculoneuropathy in children: characterized by subacuter predominantly motor dominant polyneuropathy with a favorable response to the treatment. Acta Neurol Scand 2010; 121(5): 342–347. doi: 10.1111/j.1600-0404.2009.01222.x.

7. Marsh EA, Hirst CL, Llevely JG et al. Alemtuzumab in the treatment IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol 2010; 257(6): 913–919. doi: 10.1007/s00415-009-5437-3.

8. van den Bergh PAK, Rajabally YA. Chronic inflammatory demyelinating polyradiculoneuropathy. Presse Med 2013; 42(6 Pt 2): e203–e215. doi: 10.1016/j.lpm.2013.01.056.

9. Ruts L, Drenthen J, Jacobs BC et al. Distinguishing acute-onset CIDP from fluctuating Guillain-Barré syndrome. Neurology 2010; 74(21): 1680–1686. doi: 10.1212/WNL.0b013e3181e07d14

10. Lewis RA, Sumner AJ, Brown MJ et al. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982; 32: 958–964.

11. Misra UK, Kalita J, Yadav RK. A comparison of clinical-ly atypical with typical chronic inflammatory demyelinating polyradiculoneuropathy. Eur Neurol 2007; 58(2): 100–105. doi: 10.1159/000103645.

12. Viala K, Maisonobe T, Stojkovic T et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher NervSyst 2010; 15(1): 50–56. doi: 10.1111/j.1529-8027.2010.00251.x.

13. Amato AA, Russel JA. Neuromuscular disorders. New York: McGraw Hill 2008.

14. Cocito D, Grimaldi S, Paolass I et al. Italian Network for CIDP Register. Immunsuppressive treatment in refraktory chronic inflammatory demyelinating polyradiculoneuropathy. A nationwide retrospective study. Eur J Neurol 2011; 18(12): 1417–1421. doi: 10.1111/j.1468-1331.2011.03495.x.

15. Cros D, Chiappa KH, Patel S et al. Acquired pure sensory demyelinating polyneuropathy: a chronic inflammatory polyradiculoneuropathy variant? Ann Neurol 1992; 32: 280.

16. Ohkoshi N, Harada K, Nagata H et al. Ataxic form of chronic inflammatory polyradiculoneuropathy: clinical features and pathological study of the sural nerves. Eur J Neurol 2001; 45(4): 241–248. doi: 10.1159/000052136.

17. Querol L, Nogales-GAdea G, Rojas-Garcia R et al. Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg. Neurology 2014; 82(10): 879–886. doi: 10.1212/WNL.0000000000000205.

18. Sabatelli M, Madia F, Mignogna T et al. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol 2001; 248(9): 772–777.

19. Said G, Krarup C. Chronic inflammatory demyelinating polyneuropathy. In: Said G, Kraup C (eds). Handbook of clinical neurology. Amsterdam: Elsevier 2013; 115: 404–413.

20. Ambler Z. Poruchy periferních nervů. Praha: Triton 2013.

21. Sharma KR. Demyelinating neuropathy in diabetes mellitus. Arch Neurol 2002; 59(5): 758–765.

22. Dalakas MC. Advances in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol 2011; 7(9): 507–517. doi: 10.1038/nrneurol.2011.121.

23. van den Bergh PY, Hadden RD, Rouche P et al. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy. Report of a joint task force of EFNS and PNS. J Peripher Nerv Syst 2010; 15(1): 1–9. doi: 10.1111/j.1529-8027.2010.00245.x.

24. Thaisetthawatkul P, Logigian EL, Herrmann DN. Dispersion of the distal compound musle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy. Neurology 2002; 59(10): 1526–1532.

25. Isose S, Kuwabara S, Kokubun N et al. Utility of the distal compound muscle action potential duration for diagnosis of demyelinating neuropathies. J Peripher Nerv Syst 2009; 14(3): 151–158. doi: 10.1111/j.1529-8027.2009.00226.x.

26. Isose S, Misawa S, Sonoo M et al. Duration of the distal compound muscle action potential: effects of low-cut filters. J Clin Neurophysiol 2014; 31(5): 441–443. doi: 10.1097/WNP.0000000000000045.

27. Mitsuma S, van den Bergh P, Rajabally YA et al. Effects of low frequency filtering on distal compound muscle potential duration for diagnosis of CIDP: a Japanese-European multicentre prospective study. Clin Neurophysiol 2014; 126(9): 1805–1810. doi: 10.1016/j.clinph.2014.11.027.

28. Shimizu F, Sawai S, Sano Y et al. Severity and patterns of blood-nerve barrier breakdown in patients with chronic inflammatory demyelinating polyradiculoneuropathy: correlations with clinical subtypes. PLoS ONE 2014; 9(8): e104205. doi: 10.1371/journal.pone.0104205.

29. Potulska-Chromik A, Ryniewicz B, Aragon-Gawinska K et al. An electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies. J Peripher Nerv Syst 2016; 21(1): 22–26. doi: 10.1111/jns.12152.

30. Bednařík J, Voháňka S, Ehler E et al. Standard pro léčbu pacientů s autoimunitními nervosvalovými onemocněními intravenózním lidským imunoglobulinem a plazmaferézou. Cesk Slov Neurol N 2010; 73/106(5): 579–589.

31. Hughes RAC, Donofrio P, Brill V et al. on behalf of the ICE Study Group. Intravenous immune globulin (10% caprylatechromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol 2008; 7(2): 136–144. doi: 10.1016/S1474-4422(07)70329-0.

32. Nobile-Orazio E, Cocito D, Jann S et al. Intravenous immunoglobulin versus intravenous methylprednisolone for chronic inflammatory demyelinating polyradiculoneuropahty: a randomised controlled trial. Lancet Neurol 2012; 11(6): 493–502. doi: 10.1016/S1474-4422(12)70093-5.

33. Markvardsen LH, Harbo T, Sindrup SH et al. Subcutaneous immunoglobulin preserves muscle strengthin chronic inflammatory demyelinating polyneuropathy. Eur J Neurol 2014; 21(12): 1465–1470. doi: 10.1111/ene.12080.

34. Attarian S, Verscheueren A, Franques J et al. Response to treatment in patients with Lewis Sumner syndrome. Muscle Nerve 2011; 44(2): 179–184. doi: 10.1002/mus.22024.

35. Rabin M, Mutlu G, Stojkovic T et al. Chronic inflammatory demyelinating polyradiculoneuropathy: search for factor associated with treatment dependence or successful withdrawal. J Neurol Neurosurg Psychiatry 2014; 85(8): 899–904. doi: 10.1136/jnnp-2013-306105.

36. Desai J, Ramois-Platt L, Mitchel WG. Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges, controversies and questions. Ann Indian Acad Neurol 2015; 18(3): 327–330. doi: 10.4103/0972-2327.160065.

37. Mahdi-Rogers M, van Doorn PA, Hughes RAC. Immunomodulatory treatment other than corticosteroids, immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2013; 14(6): CD003280. doi: 10.1002/14651858.CD003280.

38. Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol 2008; 65(11): 1460–1464. doi: 10.1001/archneur.65.11.1460.

39. van Schaik IN, Eftimov F, van Doorn PE et al. Pulsed high dose dexamethasone treatment versus standard prednisolone treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a double blind randomized controlled clinical trial (PREDICT study). Lancet Neurol 2010; 9(3): 245–253. doi: 10.1016/S1474-4422(10)70021-1.

40. Yoon MS, Chan A, Gold R. Standard and escalating treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Ther Adv Neurol Disor 2011; 4(3): 193–200. doi: 10.1177/1756285611405564.

41. Berger AR, Herskovitz S, Sceissa S. The restoration of IVIG efficacy by plasma Exchange in CIDP. Neurology 1995; 45(8): 1628–1629.

42. Ehler E. Chronická zánětlivá demyelinizační polyradikuloneuropatie. Neurol praxi 2015; 16(4): 215–218.

43. Katz JS, Saperstein DS, Gronseth G et al. Distal acquired demyelinating symmetric neuropathy. Neurology 2000; 54(3): 615–620.

44. Gorson KC, van Schaik IN, Merkies IS et al. Chronic inflammatory demyelinating polyneuropathy disease aktivity status: recommendations for clinical research standards and use in clinical practice. J Peripher Nerv Syst 2010; 15(4): 326–333. doi: 10.1111/j.1529-8027.2010.00284.x

45. Querol L, Rojas-Garcia R, Diaz-Manera J et al. Rituximab in treatment- resistenant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm 2015; 2(5): e149. doi: 10.1212/NXI.0000000 000000149.

46. Sommer C. Leitlinien DGN. Therapie akuter und chronischer immunvermittelter Neuropathien und Neuritiden. Stuttgart: Deutsche Gesselschaft für Neurologie 2017.