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Chronic Inflammatory Demyelinating Polyneuropathy

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Neuroimmune Diseases

Part of the book series: Contemporary Clinical Neuroscience ((CCNE))

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease that targets the myelin sheaths of peripheral nerves. Lacking a specific diagnostic test, CIDP is diagnosed on the basis of the clinical presentation and demonstration of demyelination by electrodiagnostic or nerve biopsy studies. There are many phenotype variants of CIDP. Typical CIDP involves motor and sensory nerve dysfunction, with motor deficits reported in up to 94% of patients and sensory deficits in up to 89%. Half to two-thirds of patients with CIDP display the typical phenotype. The three proven treatments for CIDP are intravenous immunoglobulin (IVIG), corticosteroids, and plasma exchange. In the last years, a set of autoantibodies against proteins located at the node of Ranvier has been identified in some patients with CIDP. IVIG treatment is not satisfactory in the majority of antibody-positive CIDP patients.

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References

  1. Harris W, Newcomb WD. A case of relapsing interstitial hypertrophic polyneuritis. Brain. 1929;52:108–16.

    Google Scholar 

  2. Austin JH. Recurrent polyneuropathies and their corticosteroid treatment; with five-year observations of a placebo-controlled case treated with corticotrophin, cortisone, and predvisone. Brain. 1958;81:157–92.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  4. Prineas JW, McLeod JG. Chronic relapsing polyneritis. J Neurol Sci. 1976;27:427–58.

    CAS  PubMed  Google Scholar 

  5. Thomas PK, Lascelles RG, Hallpike JF, Hewer RL. Recurrent and chronic relapsing Guillain-Barre polyneuritis. Brain. 1969;92:589–606.

    CAS  PubMed  Google Scholar 

  6. Mahdi-Rogers M, Hughes RA. Epidemiology of chronic inflammatory neuropathies in Southeast England. Eur J Neurol. 2014;21:28–33.

    CAS  PubMed  Google Scholar 

  7. Rajabally YA, Simpson BS, Beri S, Bankart J, Gosalakkal JA. Epidemiologic variability of chronic inflammatory demyelinating polyneuropathy with different diagnostic criteria: study of a UK population. Muscle Nerve. 2009;39:432–8.

    PubMed  Google Scholar 

  8. McLeod JG, Pollard JD, Macaskill P, Mohamed A, Spring P, Khurana V. Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales, Australia. Ann Neurol. 1999;46:910–3.

    CAS  PubMed  Google Scholar 

  9. Iijima M, Koike H, Hattori N, Tamakoshi A, Katsuno M, Tanaka F, et al. Prevalence and incidence rates of chronic inflammatory demyelinating polyneuropathy in the Japanese population. J Neurol Neurosurg Psychiatry. 2008;79:1040–3.

    CAS  PubMed  Google Scholar 

  10. Chia L, Fernandez A, Lacroix C, Adams D, Planté V, Said G. Contribution of nerve biopsy findings to the diagnosis of disabling neuropathy in the elderly. A retrospective review of 100 consecutive patients. Brain. 1996;119:1091–8.

    PubMed  Google Scholar 

  11. Suzuki M, Lacroix C, Lozeron P, Said G. Clinical features of neuropathy over 80 years: a retrospective study of 100 patients. J Neurol. 2006;253(Suppl 2):1185–6.

    Google Scholar 

  12. Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Research criteria for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology. 1991;41:617–8.

    Google Scholar 

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

    PubMed  Google Scholar 

  14. Maisonobe T, Chassande B, Vérin M, Jouni M, Léger JM, Bouche P. Chronic dysimmune demyelinating polyneuropathy: a clinical and electrophysiological study of 93 patients. J Neurol Neruosurg Psychiatry. 1996;61:36–42.

    CAS  Google Scholar 

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

    Google Scholar 

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

    Google Scholar 

  17. Dyck PJ, Tracy JA. History, diagnosis and management of chronic inflammatory demyelinating polyneuropathy. Mayo Clin Proc. 2018;93:777–93.

    PubMed  Google Scholar 

  18. Hahn AF, Hartung HP, Dyck PJ. Chronic inflammatory demyelinating polyneuropathy. In: Dyck PJ, Thomas PK, editors. Peripheral neuropathy, vol. 2. 4th ed. Philadelphia: Elsevier/Saunders; 2005. p. 2221–53.

    Google Scholar 

  19. Said G, Saimot AG, Lacroix C. Neurological complication of HIV and AIDS. In: Warlow CP, van Giin J, editors. Major problems in neurology. London: W.B. Saunders; 1997. p. 1–24.

    Google Scholar 

  20. Notermans NC, Wokke JH, Franssen H, van der Graaf Y, Vermeulen M, van den Berg LH, et al. Chronic idiopathic polyneuropathy presenting in middle or old age: a clinical and electrophysiological study of 75 patients. J Neurol Neurosurg Psychiatry. 1993;56:1066–71.

    CAS  PubMed  PubMed Central  Google Scholar 

  21. McMillan HJ, Kang PB, Jones HR, Darras BT. Childhood chronic inflammatory demyelinating polyradiculoneuropathy: combined analysis of a large cohort and eleven published series. Neuromuscul Disord. 2013;23:103–11.

    PubMed  Google Scholar 

  22. Sladky JT, Brown MJ, Berman PH. Chronic inflammatory demyelinating polyneuropathy of infancy: a corticosteroid-responsive disorder. Ann Neurol. 1986;20:76–81.

    CAS  PubMed  Google Scholar 

  23. 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.

    CAS  PubMed  Google Scholar 

  24. Figueroa JJ, Dyck PJ, Laughlin RS, Mercado JA, Massie R, Sandroni P, et al. Autonomic dysfunction in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2012;78:702–8.

    CAS  PubMed  PubMed Central  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  27. Oh SJ, Claussen GC, Kim DS. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy? J Peripher Nerv Syst. 1997;2:362–9.

    CAS  PubMed  Google Scholar 

  28. Misra VP, Walker RW. Acute-onset painful upper limb multifocal demyelinating motor neuropathy. J Neurol. 2000;247:949–54.

    CAS  PubMed  Google Scholar 

  29. Oh SJ, Joy JL, Kuruoglu R. Chronic sensory demyelinating neuropathy: chronic inflammatory demyelinating polyneuropathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992;55:677–80.

    CAS  PubMed  PubMed Central  Google Scholar 

  30. van Dijk GW, Netermans NC, Franssen H, Wokke JH. Development of weakness in patients with chronic inflammatory demyelinating polyneuropathy and only sensory symptoms at presentation: a long-term follow-up study. J Neurol. 1999;246:1134–9.

    PubMed  Google Scholar 

  31. Ohkoshi N, Harada K, Nagata H, Yato M, Shoji S. Ataxic form of chronic inflammatory demyelinating polyneuropathy: clinical features and pathological study of the sural nerves. Eur Neurol. 2001;45:241–8.

    CAS  PubMed  Google Scholar 

  32. Chin RL, Latov N, Sander HW, Hays AP, Croul SE, Magda P, et al. Sensory CIDP presenting as cry ng polyneuropathy: a study of 28 cases with nerve biopsy. J Neurol. 2003; 250 (Suppl 2):1; Ptogenic sensory polyneuropathy. J Peripher Nerv Syst. 2004; 9:132–7.

    Google Scholar 

  33. Ferreira A, Lozeron P, Lacroix C, Adams D, Said G. Sensory chronic inflammatory demyelinating polyneuropathies. J Neurol. 2003;250(Suppl 2):147.

    Google Scholar 

  34. Lozeron P, Ferreira A, Lacroix C, Adams D, Said G. Electrophysiological findings in clinical pure sensory chronic demyelinating polyneuropathies. J Neurol. 2003;250(Suppl 2):68.

    Google Scholar 

  35. Sinnreich M, Klein CJ, Daube JR, Engelstad J, Spinner RJ, Dyck PJ. Chronic immune sensory polyradiculopathy: a possible treatable sensory ataxia. Neurology. 2004;63:1662–9.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  37. Miescher GC, Latov N, Steack AJ. Dysglobulinemic neuropathies. In: Antel J, Birnbaum G, Hartung HP, editors. Clinical immunology. London: Blackwell Science; 1998. p. 307–15.

    Google Scholar 

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

    CAS  PubMed  Google Scholar 

  39. Busby M, Donaghy M. Chronic dysimmune neuropathy. A subclassification based upon the clinical feature of 102 patients. J Neurol. 2003;250:714–24.

    PubMed  Google Scholar 

  40. Korinthenberg R. Chronic inflammatory demyelinating polyradiculoneuropathy in children and their response to treatment. Neuropediatrics. 1999;30:190–6.

    CAS  PubMed  Google Scholar 

  41. Nevo Y, Pestronk A, Kornberg AJ, Connolly AM, Yee WC, Iqbal I, et al. Childhood chronic inflammatory demyelinating neuropathies: clinical course and long-term follow-up. Neurology. 1996;47:98–102.

    CAS  PubMed  Google Scholar 

  42. Hattori N, Misu K, Koike H, Ichimura M, Nagamatsu M, Hirayama M, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001;15:57–63.

    Google Scholar 

  43. Ryan MM, Grattan-Smith PJ, Procopis PG, Morgan G, Ouvrier RA. Childhood chronic inflammatory demyelinating polyneuropathy: clinical course and long-term outcome. Neuromuscul Disord. 2000;10:398–406.

    CAS  PubMed  Google Scholar 

  44. Simmons Z, Wald JJ, Albers JW. Chronic inflammatory demyelinating polyradiculoneuropathy in children: II. Long-term follow-up, with comparison to adults. Muscle Nerve. 1997;20:1569–75.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  46. Gorson KC, Ropper AH, Adelman LS, Weinberg DH. Influence of diabetes mellitus on chronic inflammatory demyelinating polyneuropathy. Muscle Nerve. 2000;23:37–43.

    CAS  PubMed  Google Scholar 

  47. Lozeron P, Nahum L, Lacroix C, Ropert A, Guglielmi JM, Said G. Symptomatic diabetic and non-diabetic neuropathies in a series of 100 diabetic patients. J Neurol. 2002;249:569–75.

    PubMed  Google Scholar 

  48. Sharma KR, Cross J, Farronay O, Ayyar DR, Shebert RT, Bradley WG. Demyelinating neuropathy in diabetes mellitus. Arch Neurol. 2002;59:758–65.

    PubMed  Google Scholar 

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

    CAS  PubMed  PubMed Central  Google Scholar 

  50. Said G, Bathien N, Cesaro P. Peripheral neuropathies and tremor. Neurology. 1982;32:480–5.

    CAS  PubMed  Google Scholar 

  51. Thomas PK, Walker RW, Rudge P, Morgan-Hughes JA, King RH, Jacobs JM, et al. Chronic demyelinating peripheral neuropathy associated with multifocal central nervous system demyelination. Brain. 1987;110:53–76.

    PubMed  Google Scholar 

  52. Mills KR, Murray NM. Neurophysiological evaluation of associated demyelinating peripheral neuropathy and multiple sclerosis: a case report. J Neurol Neurosurg Psychiatry. 1986;49:320–3.

    CAS  PubMed  PubMed Central  Google Scholar 

  53. Di Trapani G, Carnevale A, Cioffi RP, Massaro AR, Profice P. Multiple sclerosis associated with peripheral demyelinating neuropathy. Clin Neuropathol. 1996;15:135–8.

    PubMed  Google Scholar 

  54. Vural A, Doppler K, Meinl E. Autoantibodies against the node of Ranvier in seropositive chronic inflammatory demyelinating polyneuropathy: diagnostic, pathogenic, and therapeutic relevance. Front Immunol. 2018;9:1129. https://doi.org/10.3389/fimmu.2018.01029.

    Article  CAS  Google Scholar 

  55. 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.

    PubMed  Google Scholar 

  56. Van Doorn PA, Vermeulen M, Brand A, Mulder PG, Busch HF. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Clinical and laboratory characteristics associated with improvement. Arch Neurol. 1991;48:217–20.

    PubMed  Google Scholar 

  57. Hattori N, Misu K, Koike H, Ichimura M, Nagamatsu M, Hirayama M, et al. Age of onset influences clinical features of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2001;184:57–63.

    CAS  PubMed  Google Scholar 

  58. Kuwabara S, Misawa S, Mori M, Tamura N, Kubota M, Hattori T. Long term prognosis of chronic inflammatory demyelinating polyneuropathy: a five year follow up of 38 cases. J Neurol Neurosurg Psychiatry. 2006;77:66–70.

    CAS  PubMed  PubMed Central  Google Scholar 

  59. Dalakas MC. Advance in the diagnosis, pathogenesis and treatment of CIDP. Nat Rev Neurol. 2011;16:507–17.

    Google Scholar 

  60. Mathey EK, Park SB, Hughes RA, Pollard JD, Armati PJ, Barnett MH, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry. 2015;86:973–85.

    PubMed  Google Scholar 

  61. Schmit B, Toyka KV, Kiefer R, Full J, Hartung HP, Pollard J. Inflammatory infiltrates in sural nerve biopsies in Guillain-Barré syndrome and chronic inflammatory demyelinating neuropathy. Muscle Nerve. 1996;19:474–87.

    Google Scholar 

  62. Misawa S, Kuwabara S, Mori M, Kawaguchi N, Yoshiyama Y, Hattori T. Serum levels of tumor necrosis factor-alpha in chronic inflammatory demyelinating polyneuropathy. Neurology. 2001;56:666–9.

    CAS  PubMed  Google Scholar 

  63. Mathey EK, Pollard JD, Armati PJ. TNF alpha, IFN gamma and IL-2 mRNA expression in CIDP sural nerve biopsies. J Neurol Sci. 1999;163:47–52.

    CAS  PubMed  Google Scholar 

  64. Pollard JD. Chronic inflammatory demyelinating polyradiculoneuropathy. In: McLeod J, editor. Inflammatory neuropathies: Bailliere’s clinical neurology, vol. 3. London: WB Saunders; 1994. p. 107–27.

    Google Scholar 

  65. Kiefer R, Kiesseier BC, Stoll G, Hartung HP. The role of macrophages in immune-mediated damage to the peripheral nerve system. Prog Neurobiol. 2001;64:109–27.

    CAS  PubMed  Google Scholar 

  66. Mausberg AK, Dorok M, Stettner M, Muller M, Hartung HP, Dehmel T, et al. Recovery of the T-cell repertoire in CIDP by IV immunoglobulins. Neurology. 2013;80:296–303.

    CAS  PubMed  Google Scholar 

  67. Melendez-Vasquez C, Redford J, Choudhary PP, Gray IA, Maitland P, Gregson NA, et al. Immunological investigation of chronic inflammatory demyelinating polyraduculoneuropathy. J Neuroimmunol. 1997;73:124–34.

    CAS  PubMed  Google Scholar 

  68. Yuki N, Tagawa Y, Handa S. Autoantibodies to peripheral nerve glycosphingolipids SPG, SLPG and SGPG in Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 1996;70:1–6.

    CAS  PubMed  Google Scholar 

  69. Gabriel CM, Gregson NA, Hughes RA. Anti-PMP22 antibodies in patients with inflammatory neuropathy. J Neuroimmunol. 2000;104:139–46.

    CAS  PubMed  Google Scholar 

  70. 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.

    CAS  PubMed  Google Scholar 

  71. Khalili-Shirazi A, Atkinson P, Gregson N, Hughes RA. Antibody responses to P0 and P2 myelin proteins in Guillain-Barre syndrome and chronic idiopathic demyelinating polyradiculoneuropathy. J Neuroimmunol. 1993;46:245–51.

    CAS  PubMed  Google Scholar 

  72. Inglis HR, Csurhes PA, McCombe PA. Antibody responses to patients of peripheral nerve myelin proteins P0 and P2 in patients with inflammatory demyelinating neuropathy. J Neurol Neurosurg Psychiatry. 2007;78:419–22.

    CAS  PubMed  Google Scholar 

  73. Kwa MS, van Shchaik IN, Brand A, Baas F, Vermeulen M. Investigation of serum response to PMP22, connexin 32 and P0 in inflammatory neuropathies. J Neuroimmunol. 2001;116:220–5.

    CAS  PubMed  Google Scholar 

  74. Querol L, Deveax J, Rojas-Garcia R, Illa I. Autoantibodies in chronic inflammatory neuropathies: diagnostic and therapeutic implications. Nat Rev Neurol. 2017;13:533–47.

    CAS  PubMed  Google Scholar 

  75. Illa I. ARTHUR ASBURY LECTURE: chronic inflammatory demyelinating polyradiculoneuropathy: clinical aspects and new animal models of auto-immunity to nodal components. J Peripher Nerv Syst. 2017;22:418–24.

    CAS  PubMed  Google Scholar 

  76. Latov N. Diagnosis and treatment of chronic acquired demyelinating polyneuropathies. Nat Rev Neurol. 2014;10:435–46.

    CAS  PubMed  Google Scholar 

  77. 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. J Peripher Nerv Syst. 2010;15:1–9.

    Google Scholar 

  78. Hughes RA. Chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:281–2.

    CAS  PubMed  Google Scholar 

  79. Bromberg MB. Review of the evolution of electrodiagnositc criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2011;43:780–94.

    PubMed  Google Scholar 

  80. Nicolas G, Maisonobe T, Le Forestoer N, Léger JM, Bouche P. Proposed revised electrophysiological criteria for chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2002;25:26–30.

    PubMed  Google Scholar 

  81. Isose S, Kuwabara S, Kokubun N, Sato Y, Mori M, Shibuya K, et al. Utility of the distal compound muscle action potential duration for diagnosis of demyelinating neuropathies. J Peripher Nerv Syst. 2009;14:151–8.

    PubMed  Google Scholar 

  82. Kuwabara S, Ogawara K, Misawa S, Mori M, Hattori T. Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 2002;72:37–42.

    CAS  PubMed  PubMed Central  Google Scholar 

  83. Ruts L, Drenthen J, Jacobs BC, van Doom PA. Dutch GBS Study Group. Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study. Neurology. 2010;25:1680–6.

    Google Scholar 

  84. Albers JW, Kelly JJ Jr. Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve. 1989;12:435–51.

    CAS  PubMed  Google Scholar 

  85. Ho TW, Li CY, Comblath DR, Gao CY, Asbury AK, Griffin JW, et al. Patterns of recovery in the Guillain-Barre syndromes. Neurology. 1997;48:695–700.

    CAS  PubMed  Google Scholar 

  86. Hughes R, Bensa S, Willison H, Van den Bergh P, Comi G, Illa I, et al. Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:195–201.

    CAS  PubMed  Google Scholar 

  87. Bragg JA, Benatar MG. Sensory nerve conduction slowing is a specific marker for CIDP. Muscle Nerve. 2008;38:1599–603.

    PubMed  Google Scholar 

  88. Krarup C, Trojaborg W. Sensory pathophysiology in chronic acquired demyelinating neuropathy. Brain. 1996;119:257–70.

    PubMed  Google Scholar 

  89. Bromberg MB, Albers JW. Patterns of sensory nerve conduction abnormalities in demyelinating and axonal peripheral nerve disorders. Muscle Nerve. 1993;16:262–6.

    CAS  PubMed  Google Scholar 

  90. Yiannikas C, Vucic S. Utility of somatosensory evoked potentials in chronic acquired demyelinating neuropathy. Muscle Nerve. 2008;38:1447–54.

    PubMed  Google Scholar 

  91. Fuglsang-Frederiksen A, Pugdahl K. Current status on electrodiagnostic standards and guidelines in neuromuscular disorders. Clin Neurophysiol. 2011;122:440–55.

    PubMed  Google Scholar 

  92. Sander HW, Latov N. Research criteria for defining patients with CIDP. Neurology. 2003;60(Suppl 3):8–15.

    Google Scholar 

  93. Lewis RA, Sumner AJ. The electrodiagnostic distinctions between chronic familial and acquired demyelinative neuropathies. Neurology. 1982;32:592–6.

    CAS  PubMed  Google Scholar 

  94. Van den Bergh PYK, Pieret F. Electrodiagnostic criteria for acute and chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 2004;29:565–74.

    PubMed  Google Scholar 

  95. Allen JA, Lewis RA. CIDP diagnostic pitfalls and perception of treatment benefit. Neurology. 2015;85:498–504.

    CAS  PubMed  Google Scholar 

  96. Haq RU, Fries TJ, Pendlebury WW, Kenny MJ, Badger GJ, Tandan R. Chronic inflammatory demyelinating polyradiculoneuropathy: a study of proposed electrodiagnostic and histologic criteria. Arch Neurol. 2000;57:1745–50.

    CAS  PubMed  Google Scholar 

  97. Latov N. Diagnosis of CIDP. Neurology. 2002;59:S2–6.

    PubMed  Google Scholar 

  98. Vallet JM, Tabaraud F, Magy L, Torny F, Bernet-Bernady P, Macian F, et al. Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: evaluation of eight cases. Muscle Nerve. 2003;27:478–85.

    Google Scholar 

  99. Allen JA, Ney J, Lewis RA. Electrodiagnostic errors contribute to chronic inflammatory demyelinating polyneuropathy misdiagnosis. Muscle Nerve. 2018;57:542–9.

    CAS  PubMed  Google Scholar 

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

    CAS  Google Scholar 

  101. Bosboom WM, van den Berg LH, Franssen H, Giesbergen PC, Flach HZ, van Putten AM, et al. Diagnostic value of sural nerve demyelination in chronic inflammatory demyelinating polyneuropathy. Brain. 2001;124:2427–38.

    CAS  PubMed  Google Scholar 

  102. Van Es HW, van den Berg LH, Franssen H, Witkamp TD, Ramos LM, Notermans NC, et al. Magnetic resonance imaging of the brachial plexus in patients with multifocal motor neuropathy. Neurology. 1997;48:1218–24.

    PubMed  Google Scholar 

  103. Duggins AJ, McLeod JG, Pollard JD, Davies L, Yang F, Thompson EO, et al. Brain. 1999;122:1383–90.

    PubMed  Google Scholar 

  104. Hiwatashi A, Togao O, Yamashita K, Kikuchi K, Ogata H, Yamasaki R, et al. Evaluation of chronic inflammatory demyelinating polyneuropathy: 3D nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation enhancement imaging (3D SHINKEI). Eur Radiol. 2017;27:447–53.

    PubMed  Google Scholar 

  105. Ishikawa T, Asalura K, Mizutani Y, Ueda A, Murate K, Hikichi C, et al. MR neurography for the evaluation of CIDP. Muscle Nerve. 2017;55:483–9.

    PubMed  Google Scholar 

  106. Zaidman CM, Al-Lozi M, Pestrouk A. Peripheral nerve size in normal and patients with polyneuropathy: an ultrasound study. Muscle Nerve. 2009;40:960–6.

    PubMed  Google Scholar 

  107. Sugimoto T, Ochi K, Hosomi N, Takahashi T, Ueno H, Nakamura T, et al. Ultrasonographic nerve enlargement of the median an 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;260:2580–7.

    PubMed  Google Scholar 

  108. Schneider C, Bucher F, Cursiefen C, Fink GR, Heihdl LM, Lehmann HC. Corneal confocal microscopy detects small fiber damage in chronic inflammatory demyelinating polyneuropathy. J Peripher Nerv Syst. 2014;19:322–7.

    PubMed  Google Scholar 

  109. Stettner M, Hinrichs L, Guthoff R, Bairov S, Petropoulos IN, Warnke C, et al. Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy. Ann Clin Transl Neurol. 2015;3:88–100.

    PubMed  PubMed Central  Google Scholar 

  110. Prineas JW. Pathology of inflammatory demyelinating neuropathies. Baillieres Clin Neurol. 1994;3:1–24.

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  112. Rizzuto N, Morbin M, Cavallaro T, Ferrari S, Fallahi M, Galiazzo-Rizzuto S. Focal lesions area feature of chronic inflammatory demyelinating polyneuropathy (CIDP). Acta Neuropathol. 1998;96:603–9.

    CAS  PubMed  Google Scholar 

  113. Vital C, Vital A, Lagueny A, Ferrer X, Fontan D, Barat M, et al. Chronic inflammatory demyelinating polyneuropathy: immunopathological and ultrastructural study of peripheral nerve biopsy in 42 cases. Ultrastruct Pathol. 2000;24:363–9.

    CAS  PubMed  Google Scholar 

  114. Nagamatu M, Terao S, Misu K, Li M, Hattori N, Ichimura M, et al. Axonal and perikaryal involvement in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry. 1999;66:727–33.

    Google Scholar 

  115. England JD, Gronseth GS, Franklin G, Carter GT, Kinsella LJ, Cohen JA, et al. 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;72:177–84.

    CAS  PubMed  Google Scholar 

  116. Oh SJ, LaGanke C, Powers R, Wolfe GI, Quinton RA, Burns DK. Multifocal motor sensory demyelinating neuropathy: inflammatory demyelinating polyradiculoneuropathy. Neurology. 2005;65:1639–42.

    CAS  PubMed  Google Scholar 

  117. Koike H, Kadoya M, Kaida KI, Ikeda S, Kawagashira Y, Iijima M, et al. Paraodal dissection in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin-155 and anti-contactin-1 antibodies. J Neurol Neurosurg Psychiatry. 2017;88:465–73.

    PubMed  Google Scholar 

  118. Pestronk A, Cornblath DR, Ilyas AA, Baba H, Quarles RH, Griffin JW, et al. A treatable multifocal motor neuropathy with antibodies with antibodies to GM1 ganglioside. Ann Neurol. 1988;24:73–8.

    CAS  PubMed  Google Scholar 

  119. Joint Task Force of EFNS and the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society---first revision. J Peripher Nerv Syst. 2010;15:295–301.

    Google Scholar 

  120. Hahn AF, Beydoun SR, Lawson V. IVIG in MMN Study Team, Oh M, Empson VG, et al. A controlled trial of intravenous immunoglobulin in multifocal motor neuropathy. J Perpher Nerv Syst. 2013;18:321–30.

    CAS  Google Scholar 

  121. Donaghy M, Mills KR, Boniface SJ, Simmons J, Wright I, Gregson N, et al. Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry. 1994;57:778–83.

    CAS  PubMed  PubMed Central  Google Scholar 

  122. Carpo M, Cappellari A, Mora G, Pedotti R, Barbieri S, Scarlato G, et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology. 1998;50:1480–2.

    CAS  PubMed  Google Scholar 

  123. Kelly JJ. Peripheral neuropathies associated with monoclonal gammopathies of undetermined significance. Rev Neurol Dis. 2008;5:14–22.

    PubMed  Google Scholar 

  124. Dalakas MC. Advances in the diagnosis, immunopathogenesis and therapies of IgM-anti-MAG antibody-mediated neuropathies. Ther Adv Neurol Disord. 2018;11:1756285617746640.

    PubMed  PubMed Central  Google Scholar 

  125. Latov N, Hays AP, Sherman WH. Peripheral neuropathy and anti-MAG antibodies. Clit Rev Neurobiol. 1988;3:301–32.

    CAS  Google Scholar 

  126. Kaku DA, England JD, Sumner AJ. Distal accentuation of conduction slowing in polyneuropathy associated with antibodies to myelin-associated glycoprotein and sulphated glucuronyl paragloboside. Brain. 1994;117:941–7.

    PubMed  Google Scholar 

  127. Dalakas MC. Pathogenesis and treatment of anti-MAG neuropathy. Curr Treat Opt Neurol. 2010;12:71–83.

    Google Scholar 

  128. Magy L, Kabore R, Mathis S, Lebeau P, Ghorab K, Caudie C, et al. Heterogeneity of polyneuropathy associated with anti-MAG antibodies. J Immunol Res. 2015;2015:1. https://doi.org/10.1155/2015/450391.

    Article  Google Scholar 

  129. Eurelings M, Notermans NC, Van de Donk N, Lokhorst HM. Risk factors for hematological malignancy in polyneuropathy associated with monoclonal gammopathy. Muscle Nerve. 2001;24:1295–302.

    CAS  PubMed  Google Scholar 

  130. Bleasel AF, Hawke SH, Pollard JD, McLeod JG. IgG monoclonal paraproteinaemia and peripheral neuropathy. J Neurol Neurosurg Psychiatry. 1993;56:52–7.

    CAS  PubMed  PubMed Central  Google Scholar 

  131. Simmons Z, Albers JW, Bromberg MB, Feldman EL. Long-term follow-up of patients with chronic inflammatory demyelinating polyradiculoneuropathy, without and with monoclonal gammopathy. Brain. 1997;118:359–68.

    Google Scholar 

  132. Dispenzieri A. POEMS syndrome: 2017 update on diagnosis, risk stratification, and management. Am J Hematol. 2017;92:814–29.

    PubMed  Google Scholar 

  133. Keddie S, Lunn MP. POEMS syndrome. Curr Opin Neurol. 2018;31:551–8.

    CAS  PubMed  Google Scholar 

  134. Nasu S, Misawa S, Sekiguchi Y, Shibuya K, Kanai K, Fujimaki Y, et al. Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinative polyneuropathy. J Neurol Neurosurg Psychiatry. 2012;83:476–9.

    PubMed  Google Scholar 

  135. Pranté-Bordeneuve V, Ferreira A, Lalu T, Zaros C, Lacroix C, Adams D, Said G. Diagnostic pitfalls in sporadic transthyretin familial amyloid polyneuropathy (TTR-FAP). Neurology. 2007;69:693–8.

    Google Scholar 

  136. Planté-Bordeneuve V, Parman Y, Guiochon-Mantel A, Alj Y, Deymeer F, Serdaroglu P, et al. The range of chronic demyelinating polyneuropathy of infancy: a clinic-pathological and genetic study of 15 unrelated cases. J Neurol. 2001;248:795–803.

    PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  138. Bird SJ, Sladsky JT. Corticosteroid-responsive dominantly inherited neuropathy in childhood. Neurology. 1991;41:437–9.

    CAS  PubMed  Google Scholar 

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

    PubMed  Google Scholar 

  140. 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.

    CAS  PubMed  Google Scholar 

  141. Hughes RA. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyneuropathy (ICE study): a randomized placebo-controlled trial. Lancet Neurol. 2008;7:136–44.

    CAS  PubMed  Google Scholar 

  142. Dyck P, Litchy WJ, Kratz KM, Suarez GA, Low PA, Pineda AA, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinationg polyradiculoneuropathy. Ann Neurol. 1994;36:838–45.

    CAS  PubMed  Google Scholar 

  143. Van Doorn PA. Treatment of chronic inflammatory demyelinating polyneuropathy. Curr Opin Neurol. 2004;17:607–13.

    PubMed  Google Scholar 

  144. Hughes RA. Randomized controlled trial of intravenous immunoglobulin versus oral predonisilone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:195–201.

    CAS  PubMed  Google Scholar 

  145. Oaklander AL, Lunn MP, Hughes RA, van Schaik IN, Frost C, Chalk CH. Treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev. 2017:CD10369. https://doi.org/10.1002/14651858.

  146. Eftimov F, Vermeulen M, van Doorn PA, Brusse E, van Schaik IN. PREDICT. Long-term remission of CIDP after pulsed dexamethasone or short-term prednisolone treatment. Neurology. 2012;78:1079–84.

    CAS  PubMed  Google Scholar 

  147. Kazatchkine MD, Kaveri SV. Immunomodulation of autoimmune and inflammatory disease with intravenous immunoglobulin. N Engl J Med. 2001;345:747–55.

    CAS  PubMed  Google Scholar 

  148. Vermeulen M, van Doorn PA, Brand A, Strengers PF, Jennekens FG, Busch HF. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry. 1993;56:36–9.

    CAS  PubMed  PubMed Central  Google Scholar 

  149. Van Doorn PA, Brand A, Strengers PF, Meulstee J, Vermeulen M. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, crossover study. Neurology. 1990;40:209–12.

    PubMed  Google Scholar 

  150. Mendell JR, Barohn RJ, Freimer ML, Kissel JT, King W, Nagaraja HN, et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2001;56:445–9.

    CAS  PubMed  Google Scholar 

  151. van Schail IN, Bril V, van Geloven N, Hartung HP, Lewis RA, Sobue G, et al. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory in chronic inflammatory demyelinating polyneuropathy (PATH): a randomized, double-blind, placebo-controlled, phase 3 trial. Lancet Neurol. 2018;17:35–46.

    Google Scholar 

  152. Vanhoutte EK, Latov N, Deng C, Hanna K, Houghes RAC, Bril V, et al. Vigorimeter grip strength in CIDP: a responsive tool that rapidly measures the effect of IVIG and the ICE study. Eur J Neurol. 2013;20:748–55.

    CAS  PubMed  Google Scholar 

  153. Kuitwaard K, van Doorn PA. Newer therapeutic options for chronic inflammatory demyelinationg polyradiculoneuropathy. Drug. 2009;69:987–1001.

    CAS  Google Scholar 

  154. Jolles S, Orange JS, Gardulf A, Stein MR, Shapiro R, Borte M, et al. Current treatment options with immunoglobulin G for the individualization of case in patients with primary immunodeficiency disease. Clin Exp Immunol. 2015;179:146–60.

    CAS  PubMed  PubMed Central  Google Scholar 

  155. Mahdi-Rogers M, van Doorn PA, Hughes RA. Immunomodulatory treatment other than corticosteroids. Immunoglobulin and plasma exchange for chronic inflammatory demyelinating polyneuropathy. Cochrane Database Syst Rev. 2013;(14):CD003280.

    Google Scholar 

  156. Devaux JJ, Miura Y, Fukami Y, Inoue T, Manso C, Belghazi M, et al. Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy. Neurology. 2016;86:800–7.

    CAS  PubMed  PubMed Central  Google Scholar 

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Suzuki, M., Said, G. (2019). Chronic Inflammatory Demyelinating Polyneuropathy. In: Mitoma, H., Manto, M. (eds) Neuroimmune Diseases. Contemporary Clinical Neuroscience. Springer, Cham. https://doi.org/10.1007/978-3-030-19515-1_25

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