Skip to main content

Advertisement

Log in

Chronic Inflammatory Demyelinating Polyneuropathy

  • NEUROIMMUNOLOGY (RP LISAK, SECTION EDITOR)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Opinion statement

Chronic Inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progress over more than 8 weeks, being referred to as chronic inflammatory demyelinating polyneuropathy (CIDP). Despite tremendous progress in elucidating disease pathogenesis, the exact triggering event remains unknown. Our knowledge regarding diagnosis and management of CIDP and its variants continues to expand, resulting in improved opportunities for identification and treatment. Most clinical neurologists will be involved in the management of patients with these disorders, and should be familiar with available therapies for CIDP. We review the distinctive clinical, laboratory, and electro-diagnostic features that aid in diagnosis. We emphasize the importance of clinical patterns that define treatment responsiveness and the most appropriate therapies in order to improve prognosis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: •• Of major importance

  1. Dyck PJ, Oviatt KF, Lambert EH. Intensive evaluation of referred unclassified neuropathies yields improved diagnosis. Ann Neurol. 1981;10(3):222–6.

    Article  PubMed  CAS  Google Scholar 

  2. Barohn RJ. Approach to peripheral neuropathy and neuronopathy. Semin Neurol. 1998;18(1):7–18.

    Article  PubMed  CAS  Google Scholar 

  3. Khan S, Wolfe G, Nascimento O, Pasnoor M, et al. North American and South America (NA-SA) Neuropathy project (abstract). Neurology. 2006;66:A84.

    Article  Google Scholar 

  4. Pasnoor M, Nascimento O, Trivedi J, Wolfe GI, Nations S, Herbelin L, et al. North America and South America (NA-SA) Neuropathy Project. International Journal of Neuroscience 2013. doi:10.3109/00207454.2013.782026

  5. Laughlin RS, Dyck PJ, Melton 3rd LJ, Leibson C, Ransom J, Dyck PJ. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology. 2009;73(1):39–45.

    Article  PubMed  CAS  Google Scholar 

  6. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain. 1987;110:1617–30.

    Article  PubMed  Google Scholar 

  7. Holzbauer SM, DeVries AS, Sejvar JJ, et al. Epidemiologic investigation of immune-mediated polyradiculoneuropathy among abattoir workers exposed to porcine brain. PLoS One. 2010;5(3):e9782.

    Article  PubMed  CAS  Google Scholar 

  8. Dyck PJ, Arnason BG. Chronic inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, Lambert EH, Bunge R, editors. Peripheral neuropathy. 2nd ed. Philadelphia: WB Saunders; 1984. p. 2101–14.

    Google Scholar 

  9. Austin JH. Recurrent polyneuropathies and their corticosteroids treatment. Brain. 1958;8:157–92.

    Article  Google Scholar 

  10. Viala K, Maisonobe T, Stojkovic T, Koutlidis R, Ayrignac X, Musset L, et al. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2010;15(1):50–6.

    Article  PubMed  CAS  Google Scholar 

  11. Gorson KC, Allam G, Ropper AH. Chronic inflammatory demyelinating polyneuropathy: clinical features and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology. 1997;48:321–8.

    Article  PubMed  CAS  Google Scholar 

  12. Sabatelli M, Madia F, Mignogna T, Lippi G, Quaranta L, Tonali P. Pure motor chronic inflammatory demyelinating polyneuropathy. J Neurol. 2001;248:772–7.

    Article  PubMed  CAS  Google Scholar 

  13. Dionne A, Nicolle MW, Hahn AF. Clinical and electrophysiological parameters distinguishing acute-onset chronic inflammatory demyelinating polyneuropathy from acute inflammatory demyelinating polyneuropathy. Muscle Nerve. 2010;41(2):202–7.

    PubMed  Google Scholar 

  14. Ruts L, Drenthen J, Jacobs BC, van Doorn PA, Dutch GBS Study Group. Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study. Neurology. 2010;74(21):1680–6.

    Article  PubMed  CAS  Google Scholar 

  15. Hughes R, Sanders E, Hall S, Atkinson P, Colchester A, Payan P. Subacute idiopathic demyelinating polyradiculoneuropathy. Arch Neurol. 1992;49:612–6.

    Article  PubMed  CAS  Google Scholar 

  16. Oh SJ. Subacute demyelinating polyneuropathy responding to corticosteroid treatment. Arch Neurol. 1978;35:509–16.

    Article  PubMed  CAS  Google Scholar 

  17. Oh SJ, Kurokawa K, de Almeida DF, Ryan HF, Claussen GC. Subacute inflammatory demyelinating polyneuropathy. Neurology. 2003;61:1507–12.

    Article  PubMed  CAS  Google Scholar 

  18. Saperstein DS, Katz JS, Amato AA, Barohn RJ. Clinical spectrum of chronic acquired demyelinating polyneuropathies. Muscle Nerve. 2001;24:311–24.

    Article  PubMed  CAS  Google Scholar 

  19. Koller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005;352:1343–56.

    Article  PubMed  Google Scholar 

  20. Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ. Distal acquired demyelinating symmetric (DADS) neuropathy. Neurology. 2000;54:615–20.

    Article  PubMed  CAS  Google Scholar 

  21. Saperstein DS, Amato AA, Wolfe GI, Katz JS, Nations SP, Jackson CE, et al. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve. 1999;22:560–6.

    Article  PubMed  CAS  Google Scholar 

  22. Viala K, Renié L, Maisonobe T, et al. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004;127:2010–7.

    Article  PubMed  CAS  Google Scholar 

  23. Verschueren A, Azulay JP, Attarian S, et al. Lewis-Sumner syndrome and multifocal motor neuropathy. Muscle Nerve. 2004;31:88–94.

    Article  Google Scholar 

  24. Alaedini A, Sanders HW, Hays AP, Latov N. Antigangioside antibodies in multifocal acquired sensory and motor neuropathy. Arch Neurol. 2003;60:42–6.

    Article  PubMed  Google Scholar 

  25. Oh SJ, Claussen GC, Dae SK. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate variant of chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 1997;2:362–9.

    PubMed  CAS  Google Scholar 

  26. Gorson KC, Ropper AH, Weinberg DH. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 1999;22:758–65.

    Article  PubMed  CAS  Google Scholar 

  27. Thomas PK, Claus D, Jaspert A, Workman JM, King RH, Larner AJ, et al. Focal upper limb demyelinating neuropathy. Brain. 1996;119:765–74.

    Article  PubMed  Google Scholar 

  28. Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology. 1982;32:958–64.

    Article  PubMed  CAS  Google Scholar 

  29. Gibbels E, Behse F, Kentenich M, Haupt WF. Chronic multifocal neuropathy with persistent conduction block (Lewis- Sumner syndrome). A clinico-morphologic study of two further cases with review of the literature. Clin Neuropathol. 1993;12:343–52.

    PubMed  CAS  Google Scholar 

  30. Nukada H, Pollock M, Haas LF. Is ischemia implicated in chronic multifocal demyelinating neuropathy? Neurology. 1989;39:106–10.

    Article  PubMed  CAS  Google Scholar 

  31. Dyck PJ, Lais AC, Otha M, Bastron JA, Okasaki H, Groover RV. Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc. 1975;50:621–37.

    PubMed  CAS  Google Scholar 

  32. Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989;46:878–84.

    Article  PubMed  CAS  Google Scholar 

  33. Dalakas M, Houff SA, Engel WK, Madden DL, Sever JL. CSF "monoclonal" bands in chronic relapsing polyneuropathy. Neurology. 1980;30:864–7.

    Article  PubMed  CAS  Google Scholar 

  34. Bromberg MB. Comparison of electrodiagnostic criteria for primary demyelination in chronic polyneuropathy. Muscle Nerve. 1991;14:968–76.

    Article  PubMed  CAS  Google Scholar 

  35. Thaisetthawatkul P, Logigian EL, Herrmann DN. Dispersion of the distal compound muscle action potential as a diagnostic criterion for chronic inflammatory demyelinating polyneuropathy. Neurology. 2002;59(10):1526–32.

    Article  PubMed  Google Scholar 

  36. Van den Bergh PY, Pieret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2004;29(4):565–74.

    Article  PubMed  Google Scholar 

  37. Dimachkie MM, Anderson HS, Gronseth GS, Herbelin L, Pasnoor M, McVey AL, et al. How common is sural nerve sparing in chronic inflammatory demyelinating polyneuropathy? 2008 meeting of the Inflammatory Neuropathy Consortium. JPNS. 2008;13:166a.

    Google Scholar 

  38. Koski CL, Baumgarten M, Magder LS, Barohn RJ, Goldstein J, Graves M, et al. Derivation and validation of diagnostic criteria for chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2009;277(1-2):1–8.

    Article  PubMed  CAS  Google Scholar 

  39. Van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, et al. European Federation of Neurological Societies; Peripheral Nerve Society European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol. 2010;17(3):356–63. This report expands the electrophysiologic criteria and clinical phenotypes of CIDP. It also updates treatment recommendations.

    Article  PubMed  Google Scholar 

  40. Rajabally YA, Samarasekera S. Electrophysiological sensory demyelination in typical chronic inflammatory demyelinating polyneuropathy. Eur J Neurol. 2010;17(7):939–44.

    Article  PubMed  CAS  Google Scholar 

  41. Molenaar DS, Vermeulen M, de Haan R. Diagnostic value of sural nerve biopsy in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1998;64:84–9.

    Article  PubMed  CAS  Google Scholar 

  42. Bosboom WM, Van den Berg LH, Franssen H, et al. Diagnostic value of sural demyelination in chronic inflammatory demyelinating polyneuropathy. Brain. 2001;124:2427–38.

    Article  PubMed  CAS  Google Scholar 

  43. Sommer C, Koch S, Lammens M, Gabreels-Festen A, Stoll G, Toyka KV. Macrophage clustering as a diagnostic marker in sural nerve biopsies of patients with CIDP. Neurology. 2005;65(12):1924–9.

    Article  PubMed  CAS  Google Scholar 

  44. England JD, Gronseth GS, Franklin G, Carter GT, Kinsella LJ, Cohen JA, et al. American Academy of Neurology. Practice Parameter: evaluation of distal symmetric polyneuropathy: role of autonomic testing, nerve biopsy, and skin biopsy (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation. Neurology. 2009;72(2):177–84.

    Article  PubMed  CAS  Google Scholar 

  45. Hafer-Macko CE, Sheikh KA, Li CY, Ho TW, Cornblath DR, McKhann GM, et al. Immune attack on the Schwann cell surface in acute inflammatory demyelinating polyneuropathy. Ann Neurol. 1996;39(5):625–35.

    Article  PubMed  CAS  Google Scholar 

  46. Matsummuro K, Izumo S, Umehara F, Osame M. Chronic inflammatory demyelinating polyneuropathy: Histological and immunopathological studies in biopsied sural nerves. J Neurol Sci. 1994;127:170–8.

    Article  Google Scholar 

  47. Kieseier BC, Tani M, Mahad D, Oka N, Ho T, Woodrofe N, et al. Chemokines and chemokine receptors in inflammatory demyelinating neuropathies: a central role for IP-10. Brain. 2002;125:823–34.

    Article  PubMed  Google Scholar 

  48. Leppert D, Hughes P, Huber S, Erne B, Grygar C, Said G, et al. Matrix metalloproteinase upregulation in chronic inflammatory demyelinating polyneuropathy and nonsystemic vasculitic neuropathy. Neurology. 1999;53:62–70.

    Article  PubMed  CAS  Google Scholar 

  49. Yan WX, Taylor J, Andrias-Kauba S, Pollard JD. Passive transfer of demyelination by serum or IgG from chronic inflammatory demyelinating polyneuropathy patients. Ann Neurol. 2000;47:765–75.

    Article  PubMed  CAS  Google Scholar 

  50. Koski CL, Sanders ME, Swoveland PT, et al. Activation of terminal components of complement in patients with Guillain-Barre syndrome and other demyelinating neuropathies. J Clin Invest. 1987;80:1492–7.

    Article  PubMed  CAS  Google Scholar 

  51. Hays AP, Lee SS, Latov N. Immune reactive C3d on the surface of myelin sheaths in neuropathy. J Neuroimmunol. 1988;18:231–44.

    Article  PubMed  CAS  Google Scholar 

  52. Allen D, Giannopoulos K, Gray I, Gregson N, Makowska A, Pritchard J, et al. Antibodies to peripheral nerve myelin proteins in chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2005;10(2):174–80.

    Article  PubMed  CAS  Google Scholar 

  53. De Luca G, Lugaresi A, Iarlori C, et al. Prednisone and plasma exchange improve suppressor cell function in chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 1999;95:190–4.

    Article  PubMed  Google Scholar 

  54. Chi LJ, Wang HB, Wang WZ. Impairment of circulating CD4 + CD25+ regulatory T cells in patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Peripher Nerv Syst. 2008;13:54–63.

    Article  PubMed  CAS  Google Scholar 

  55. Nimmerjahn F, Lünemann JD. Expression and function of the inhibitory Fcγ-receptor in CIDP. J Peripher Nerv Syst. 2011;16 Suppl 1:41–4.

    Article  PubMed  CAS  Google Scholar 

  56. Tackenberg B, Jelcic I, Baerenwaldt A, Oertel WH, Sommer N, Nimmerjahn F, et al. Impaired inhibitory Fcgamma receptor IIB expression on B cells in chronic inflammatory demyelinating polyneuropathy. Proc Natl Acad Sci U S A. 2009;106:4788–92.

    Article  PubMed  CAS  Google Scholar 

  57. Comi C, Gaviani P, Leone M, Ferretti M, Castelli L, Mesturini R, et al. Fas-mediated T-cell apoptosis is impaired in patients with chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 2006;11(1):53–60.

    Article  PubMed  CAS  Google Scholar 

  58. Amato AA, Barohn RJ, Sahenk Z, Tushka PJ, Mendell JR. Polyneuropathy complicating bone marrow transplantation. Neurology. 1993;43:1513–8.

    Article  PubMed  CAS  Google Scholar 

  59. Rechthand E, Cornblath DR, Stern BJ, Meyerhoff JO. Chronic demyelinating polyneuropathy in systemic lupus erythematosus. Neurology. 1984;34:1375–7.

    Article  PubMed  CAS  Google Scholar 

  60. Romanick-Schmiedl S, Kiprov DD, Chalmers AC, Miller RG. Extraneural manifestations of chronic inflammatory demyelinating polyradiculoneuropathy. Am J Med. 1990;89:531–4.

    Article  PubMed  CAS  Google Scholar 

  61. Stewart JD, McKelvey R, Durcan L, Carpenter S, Karpati G. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci. 1996;142:59–64.

    Article  PubMed  CAS  Google Scholar 

  62. Taylor BV, Wijdicks EFM, Poterucha JJ, Weinser RH. Chronic inflammatory demyelinating polyneuropathy complicating liver transplantation. Ann Neurol. 1995;38:828–31.

    Article  PubMed  CAS  Google Scholar 

  63. Dyck PJ, Swanson CJ, Low PA, Bartleson JD, Lambert EH. Prednisone-responsive hereditary motor and sensory neuropathy. Mayo Clin Proc. 1982;57:239–46.

    PubMed  CAS  Google Scholar 

  64. Mendell JR, Kolkin S, Kissel JT, Weiss KL, Chakeres DW, Rammohan KW. Evidence for central nervous system demyelination in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1987;37(8):1291–4.

    Article  PubMed  CAS  Google Scholar 

  65. Ad Hoc Subcommittee of the American Academy of Neurology. AIDS Task Force. Research criteria for diagnosis chronic inflammatory demyelinating polyneuropathy. Neurology. 1991;41:617–8.

    Article  Google Scholar 

  66. Ginsberg L, Malik O, Kenton AR, Sharp D, Muddle JR, Davis MB, et al. Coexistent hereditary and inflammatory neuropathy. Brain. 2004;127(Pt 1):193–202.

    Article  PubMed  Google Scholar 

  67. Jann S, Bramerio MA, Facchetti D, Sterzi R. Intravenous immunoglobulin is effective in patients with diabetes and with chronic inflammatory demyelinating polyneuropathy: long term follow-up. J Neurol Neurosurg Psychiatry. 2009;80(1):70–3.

    Article  PubMed  CAS  Google Scholar 

  68. Kalita J, Misra UK, Yadav RK. A comparative study of chronic inflammatory demyelinating polyradiculoneuropathy with and without diabetes mellitus. Eur J Neurol. 2007;14(6):638–43.

    Article  PubMed  CAS  Google Scholar 

  69. Stübgen JP. Tumor necrosis factor-alpha antagonists and neuropathy. Muscle Nerve. 2008;37(3):281–92.

    Article  PubMed  CAS  Google Scholar 

  70. Kundi S, Barohn R, McVey A, Dimachkie M, Pasnoor M, Herbelin L. Tacrolimus and Relapsing-Remitting CIDP: a case report. J Clin Neuromuscul Dis. 2010;11(3):9.

    Google Scholar 

  71. Wilson JR, Conwit RA, Eidelman BH, Starzl T, Abu-Elmagd K. Sensorimotor neuropathy resembling CIDP in patients receiving FK506. Muscle Nerve. 1994;17(5):528–32.

    Article  PubMed  CAS  Google Scholar 

  72. Echaniz-Laguna A, Battaglia F, Ellero B, Mohr M, Jaeck D. Chronic inflammatory demyelinating polyradiculoneuropathy in patients with liver transplantation. Muscle Nerve. 2004;30(4):501–4.

    Article  PubMed  Google Scholar 

  73. Labate A, Morelli M, Palamara G, Pirritano D, Quattrone A. Tacrolimus-induced polyneuropathy after heart transplantation. Clin Neuropharmacol. 2010;33(3):161–2.

    Article  PubMed  CAS  Google Scholar 

  74. Rotta FT, Sussman AT, Bradley WG, Ayyar DR, Sharma KR, Shebert RT. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2000;173:129–39.

    Article  PubMed  CAS  Google Scholar 

  75. Kelly JJ, Kyle RA, Miles JM, Dyck PJ. Osteosclerotic myeloma and peripheral neuropathy. Neurology. 1983;33(2):202–10.

    Article  PubMed  Google Scholar 

  76. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101(7):2496–506.

    Article  PubMed  CAS  Google Scholar 

  77. Watanabe O, Maruyama I, Arimura K, Kitajima I, Arimura H, Hanatani M, et al. Overproduction of vascular endothelial growth factor - Vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve. 1998;21(11):1390–7.

    Article  PubMed  CAS  Google Scholar 

  78. Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet. 1996;347(9002):702.

    Article  PubMed  CAS  Google Scholar 

  79. Dyck PJ, O'Brien PC, Oviatt KF, Dinapoli RP, Daube JR, Bartleson JD, et al. Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol. 1982;11:136–41.

    Article  PubMed  CAS  Google Scholar 

  80. Dyck PJ, Daube J, O'Brien P, Pineda A, Low PA, Windebank AJ, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. N Engl J Med. 1986;314:461–5.

    Article  PubMed  CAS  Google Scholar 

  81. Hahn AF, Bolton CF, Pillay N, Chalk C, Benstead T, Bril V, et al. Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy: a double-blind, sham-controlled, cross-over study. Brain. 1996;119:1055–66.

    Article  PubMed  Google Scholar 

  82. Hahn AF, Bolton CF, Zochodne D, Feasby TE. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, cross-over study. Brain. 1996;119:1067–77.

    Article  PubMed  Google Scholar 

  83. Mendell JR, Barohn RJ, Freimer ML, Kissel JT, King W, Nagaraja HN, et al. Working group on peripheral neuropathy: randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001;56:445–9.

    Article  PubMed  CAS  Google Scholar 

  84. Hughes RA, Donofrio P, Bril V, Dalakas MC, Deng C, Hanna K, et al. ICE Study Group. Intravenous immune globulin (10 % caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomized placebo-controlled trial. Lancet Neurol. 2008;7(2):136–44.

    Article  PubMed  CAS  Google Scholar 

  85. Hughes R, Bensa S, Willison H, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:195–201.

    Article  PubMed  CAS  Google Scholar 

  86. Lopate G, Pestronk A, Al-Lozi M. Treatment of chronic inflammatory demyelinating polyneuropathy with high-dose intermittent intravenous methylprednisolone. Arch Neurol. 2005;62:249–54.

    Article  PubMed  Google Scholar 

  87. Muley SA, Kelkar P, Parry GJ. Treatment of chronic inflammatory demyelinating polyneuropathy with pulsed oral steroids. Arch Neurol. 2008;65(11):1460–4.

    Article  PubMed  Google Scholar 

  88. Harbo T, Andersen H, Jakobsen J. Acute motor response following a single IVIG treatment course in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2009;39(4):439–47.

    Article  PubMed  CAS  Google Scholar 

  89. Bril V, Banach M, Dalakas MC, Deng C, Donofrio P, Hanna K, et al. ICE Study Group. Electrophysiologic correlations with clinical outcomes in CIDP. Muscle Nerve. 2010;42(4):492–7.

    Article  PubMed  Google Scholar 

  90. Lee DH, Linker RA, Paulus W, Schneider-Gold C, Chan A, Gold R. Subcutaneous immunoglobulin infusion: a new therapeutic option in chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2008;37(3):406–9.

    Article  PubMed  CAS  Google Scholar 

  91. Kaneko Y, Nimmerjahn F, Ravetch JV. Anti-inflammatory activity of immunoglobulin G resulting from Fc sialylation. Science. 2006;313(5787):670–3.

    Article  PubMed  CAS  Google Scholar 

  92. Dyck PJ, O'Brien PC, Swanson C, et al. Combined azathioprine and prednisone in chronic inflammatory-demyelinating polyneuropathy. Neurology. 1985;35:1173–6.

    Article  PubMed  CAS  Google Scholar 

  93. Hodgkinson SJ, Pollard JD, McLeod JG. Cyclosporine A in the treatment of chronic demyelinating polyradiculopathy. J Neurol Neurosurg Psychiatry. 1990;53:327–30.

    Article  PubMed  CAS  Google Scholar 

  94. Barnett MH, Pollard JD, Davies L, McLeod JG. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1998;21(4):454–60.

    Article  PubMed  CAS  Google Scholar 

  95. Koski CL. Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy: pathogenesis and treatment. Semin Neurol. 1994;14:123–30.

    Article  PubMed  CAS  Google Scholar 

  96. Good JL, Chehrenama M, Mayer RF, Koski CL. Pulsed cyclophosphamide in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998;51:1735–8.

    Article  PubMed  CAS  Google Scholar 

  97. Brannagan TH, Pradham A, Heiman-Patterson T, et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology. 2002;58:1856–8.

    Article  PubMed  CAS  Google Scholar 

  98. Chaudhry V, Cornblath DR, Griffin JW, O'Brien R, Drachman DB. Mycophenolate mofetil: a safe and promising immunosuppressant in neuromuscular diseases. Neurology. 2001;56:94–6.

    Article  PubMed  CAS  Google Scholar 

  99. Mowzoon N, Sussman A, Bradley WG. Mycophenolate (CellCept) treatment of myasthenia gravis, chronic inflammatory polyneuropathy, and inclusion body myositis. J Neurol Sci. 2001;185(2):119–22.

    Article  PubMed  CAS  Google Scholar 

  100. Gorson KC, Amato AA, Ropper AH. Efficacy of mycophenolate mofetil in patients with chronic immune demyelinating polyneuropathy. Neurology. 2004;63:715–7.

    Article  PubMed  CAS  Google Scholar 

  101. Sabatelli M, Mignona T, Lippi G, Milone M, Lazzaro VD, Tonali P. Interferon-alpha may benefit steroid unresponsive chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1995;58:638–9.

    Article  PubMed  CAS  Google Scholar 

  102. Choudhary PP, Thompson N, Hughes RAC. Improvement following interferon beta in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol. 1995;242:252–3.

    Article  PubMed  CAS  Google Scholar 

  103. Gorson KC, Ropper AH, Clark BD, Dew RB, Simovic D, Allam G. Treatment of chronic inflammatory demyelinating polyneuropathy with interferon alpha 2a. Neurology. 1998;50:84–7.

    Article  PubMed  CAS  Google Scholar 

  104. Hadden RDM, Sharrack B, Bensa S, Soudain SE, Hughes RAC. Randomized trial of interferon B-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999;53:57–61.

    Article  PubMed  CAS  Google Scholar 

  105. Kuntzer T, Radziwill AJ, Lettry-Trouillat R, et al. Interferon-B1a in chronic inflammatory demyelinating polyneuropathy. Neurology. 1999;53:1364–5.

    Article  PubMed  CAS  Google Scholar 

  106. Pavesi G, Cattaneo L, Marbini A, Gemignani F, Mancia D. Long-term efficacy of interferon-alpha in chronic inflammatory demyelinating neuropathy. J Neurol. 2002;249:777–9.

    Article  PubMed  Google Scholar 

  107. Vallat JM, Hahn AF, Leger JM, et al. Interferon beta 1a as an investigational treatment for CIDP. Neurology. 2003;60 Suppl 3:S23–8.

    Article  PubMed  CAS  Google Scholar 

  108. Fialho D, Chan YC, Allen DC, Reilly MM, Hughes RA. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate. J Neurol Neurosurg Psychiatry. 2006;77:544–7.

    Article  PubMed  CAS  Google Scholar 

  109. RMC Trial Group. Randomized controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC trial): a pilot, multicenter study. Lancet Neurol. 2009;8(2):158–64.

    Article  CAS  Google Scholar 

  110. Hughes RA, Gorson KC, Cros D, Griffin J, Pollard J, Vallat JM, et al. Avonex CIDP Study Group. Intramuscular interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2010;74(8):651–7.

    Article  PubMed  CAS  Google Scholar 

  111. Niermeijer JM, Eurelings M, Lokhorst H, Franssen H, Fijnheer R, Wokke JH, et al. Neurologic and hematologic response to fludarabine treatment in IgM MGUS polyneuropathy. Neurology. 2006;67(11):2076–9.

    Article  PubMed  CAS  Google Scholar 

  112. Gladstone DE, Prestrud AA, Brannagan III TH. High-dose cyclophosphamide results in long-term disease remission with restoration of a normal quality of life in patients with severe refractory chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 2005;10(1):11–6.

    Article  PubMed  CAS  Google Scholar 

  113. Benedetti L, Briani C, Franciotta D, Fazio R, Paolasso I, Comi C, et al. Rituximab in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a report of 13 cases and review of the literature. J Neurol Neurosurg Psychiatry. 2011;82(3):306–8.

    Article  PubMed  CAS  Google Scholar 

  114. Marsh EA, Hirst CL, Llewelyn JG, Cossburn MD, Reilly MM, Krishnan A, et al. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010;257(6):913–9.

    Article  PubMed  CAS  Google Scholar 

  115. Gordon PH, Brannagan TH, Latov N. Neurological manifestations of paraproteinemia and cryoglobulinemia. In: Aminoff MJ, Goetz CG, editors. Handbook of clinical neurology. Science.: Elsevier; 1998. p. 431–59.

    Google Scholar 

  116. Oyama R MD, Sufit MDY, Loh MDL, Statkute MDK, Yaung RNK, Quigley RNE, et al. Nonmyeloablative autologous hematopoietic stem cell transplantation for refractory CIDPY. Neurology. 2007;69:1802–3.

    Article  PubMed  CAS  Google Scholar 

  117. Kuwabara S, Misawa S, Kanai K, Kikkawa Y, Nishimura M, Nakaseko C, et al. Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology. 2006;66(1):105–7.

    Article  PubMed  CAS  Google Scholar 

  118. Mahdi-Rogers M, Kazmi M, Ferner R, Hughes RA, Renaud S, Steck AJ, et al. Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. J Peripher Nerv Syst. 2009;14(2):118–24.

    Article  PubMed  Google Scholar 

  119. Kuwabara S, Misawa S, Kanai K, Sawai S, Hattori T, Nishimura M, et al. Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. J Neurol Neurosurg Psychiatry. 2008;79(11):1255–7.

    Article  PubMed  CAS  Google Scholar 

  120. Hughes RA, Bouche P, Cornblath DR, Evers E, Hadden RD, Hahn A, et al. In: European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society. Eur J Neurol. 2006;13(4):326–32.

    Article  PubMed  CAS  Google Scholar 

  121. Bouchard C, Lacroix C, Plante V, Adams D, Chedru F, Guglielmi JM, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999;52:498–503.

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest

Mazen M. Dimachkie declares that he has no conflict of interest.

Richard J. Barohn declares that he has no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mazen M. Dimachkie M.D..

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dimachkie, M.M., Barohn, R.J. Chronic Inflammatory Demyelinating Polyneuropathy. Curr Treat Options Neurol 15, 350–366 (2013). https://doi.org/10.1007/s11940-013-0229-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11940-013-0229-6

Keywords

Navigation