Autoimmune Neuropathies

  • Jacques Reynolds
  • George SachsEmail author
Part of the Current Clinical Neurology book series (CCNEU)


Guillain-Barré syndrome and CIDP, the most common autoimmune neuropathies, can reflect a variety of underlying pathophysiologic mechanisms. This chapter reviews classic presentations of these disorders along with their variants. Clinical features, electrodiagnostic findings, and specific antibody profiles are discussed with regard to treatment options and prognosis. Multifocal motor neuropathy and neuropathies associated with monoclonal gammopathies are also reviewed.


Neuropathy Guillain-Barré CIDP Campylobacter Monoclonal gammopathy 


  1. 1.
    Guillain G, Barré JA, Strohl A. Sur un syndrome de radiculonévrite avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire. Remarques sur les caractéres cliniques et graphiques des réflexes tendineux. Bull Soc Méd Hôp Paris. 1916;40:1462–70.Google Scholar
  2. 2.
    Guillain G. Considerations sur le syndrome de Guillain-Barré. Ann Med Interne. 1953;54:81–149.Google Scholar
  3. 3.
    Waksman BH, Adams RD. Allergic neuritis: experimental disease of rabbits induced by the injection of peripheral nervous tissue and adjuvants. J Exp Med. 1955;102:213–21.PubMedPubMedCentralCrossRefGoogle Scholar
  4. 4.
    Asbury AK, Arnason BG, Adams RD. The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis. Medicine. 1969;48:173–215.PubMedCrossRefPubMedCentralGoogle Scholar
  5. 5.
    Haymaker W, Kernohan JW. The Landry Guillain Barre Syndrome: a clinicopathologic report of fifty fatal cases and a critique of the literature. Medicine. 1949;28:59–68.PubMedCrossRefPubMedCentralGoogle Scholar
  6. 6.
    Ropper AH. The Guillain-Barre syndrome. N Engl J Med. 1992;326:1130.PubMedCrossRefPubMedCentralGoogle Scholar
  7. 7.
    Gorson KC, Ropper AH. Guillain-Barre syndrome (acute inflammatory demyelinating polyneuropathy) and related disorders. In: Katirji B, Kaminski HJ, Preston DC, Ruff RL, Shapiro BE, editors. Neuromuscular disorders in clinical practice. Boston: Butterworth Heinemann; 2002. p. 544–66.Google Scholar
  8. 8.
    Thaisetthawatkul P, Logigian EL. Peripheral nervous system manifestations of lyme borreliosis. J Clin Neuromuscul Dis. 2002;3(4):165–71.PubMedCrossRefPubMedCentralGoogle Scholar
  9. 9.
    Albers JW, Donofrio PD, McGonagle TK. Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1985;6:504–9.Google Scholar
  10. 10.
    Cornblath DR. Electrophysiology in Guillain-Barré syndrome. Ann Neurol. 1990;27(Suppl):S17–20.PubMedCrossRefPubMedCentralGoogle Scholar
  11. 11.
    Cornblath MP, Mellits ED, Griffin JW, et al. Motor conduction studies in Guillain-Barre syndrome: description and prognostic value. Ann Neurol. 1988;23:354–9.PubMedCrossRefPubMedCentralGoogle Scholar
  12. 12.
    Deepak G, Muraleedharan N, Baheti NN, Sarma SP, Abraham K. Electrodiagnostic and clinical aspects of Guillain-Barré syndrome: an analysis of 142 cases. J Clin Neuromuscul Dis. 2008;10(2):42–51.PubMedCrossRefPubMedCentralGoogle Scholar
  13. 13.
    Prineas JW. Acute idiopathic polyneuritis. An electron microscope study. Lab Investig. 1972;26:133–47.PubMedPubMedCentralGoogle Scholar
  14. 14.
    McKhann GM, Cornblath DR, Griffin JW, et al. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol. 1993;33(4):333–42.PubMedCrossRefPubMedCentralGoogle Scholar
  15. 15.
    Hafer-Macko C, Hsieh ST, Li CY, et al. Acute motor axonal neuropathy: an antibody-mediated attack on axolemma. Ann Neurol. 1996;40:635–44.PubMedCrossRefPubMedCentralGoogle Scholar
  16. 16.
    White JR, Sachs GM, Gilchrist J. Multifocal motor neuropathy with conduction block and Campylobacter jejuni. Neurology. 1996;46(2):562–3.PubMedCrossRefPubMedCentralGoogle Scholar
  17. 17.
    Abruzzese M, Reni L, Schenone A, Mancardi GL, Primavera A. Multifocal motor neuropathy with conduction block after Campylobacter jejuni enteritis. Neurology. 1997;48:544.CrossRefGoogle Scholar
  18. 18.
    Fisher M. An unusual variant of acute immune polyneuritis (syndrome of ophthalmoplegia, ataxia, and areflexia). N Engl J Med. 1956;255:57–65.PubMedCrossRefPubMedCentralGoogle Scholar
  19. 19.
    Guiloff RJ. Peripheral nerve conduction in Miller Fisher syndrome. J Neurol Neurosurg Psychiatry. 1977;40:801–7.PubMedPubMedCentralCrossRefGoogle Scholar
  20. 20.
    Fross RD, Daube J. Neuropathy in the Miller-Fisher syndrome: clinical and electrophysiologic findings. Neurology. 1987;37:1493–8.PubMedCrossRefPubMedCentralGoogle Scholar
  21. 21.
    Wietholder H, Hulser PJ, Meier DH, Wessel K. Electrophysiological follow up of experimental allergic neuritis mediated by permanent T cell lines in rats. J Neurol Sci. 1988;83:1–12.CrossRefGoogle Scholar
  22. 22.
    Hughes RA, Cornblath DR. Guillain Barre syndrome. Lancet. 2005;366:1653–66.PubMedCrossRefPubMedCentralGoogle Scholar
  23. 23.
    Ogawara K, Kuwabara S, Mori M, et al. Axonal Guillain Barre syndrome: relation to antiganglioside antibodies and Campylobacter jejuni infection in Japan. Ann Neurol. 2000;48:624–31.PubMedCrossRefPubMedCentralGoogle Scholar
  24. 24.
    Gong Y, Tagawa Y, Lunn MP, et al. Localization of major gangliosides in the PNS. Brain. 2002;125:2491–9.PubMedCrossRefPubMedCentralGoogle Scholar
  25. 25.
    Yoshino H. Distribution of gangliosides in the nervous tissues recognized by axonal form of Guillain Barre syndrome. Neuroimmunology. 1997;5:174–82.Google Scholar
  26. 26.
    Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Ganglioside composition of the human cranial nerves, with special reference to pathophysiology of Miller Fisher syndrome. Brain Res. 1997;745:32–6.PubMedCrossRefPubMedCentralGoogle Scholar
  27. 27.
    Notturno F, Caporale CM, Uncini A. Acute sensory ataxic neuropathy with antibodies to GD1b and GQ1b gangliosides and prompt recovery. Muscle Nerve. 2008;37:265–8.PubMedCrossRefPubMedCentralGoogle Scholar
  28. 28.
    Nagashima T, Koga M, Odaka M, Hirata K, Yuki N. Clinical correlates of serum anti-GT1a IgG antibodies. J Neurol Sci. 2004;219:139–45.PubMedCrossRefPubMedCentralGoogle Scholar
  29. 29.
    Susuki K, Nishimoto Y, Yamada M, et al. Acute motor axonal neuropathy rabbit model: immune attack on nerve root axons. Ann Neurol. 2003;54:383–8.PubMedCrossRefPubMedCentralGoogle Scholar
  30. 30.
    Yuki N, Yamada M, Koga M, et al. Animal model of axonal Guillain-Barré syndrome induced by sensitization with GM1 ganglioside. Ann Neurol. 2001;49:712–20.PubMedCrossRefPubMedCentralGoogle Scholar
  31. 31.
    Illa I, Ortiz N, Gallard E, Juarez C, Grau JM, Dalakas MC. Acute axonal Guillain-Barré syndrome with IgG antibodies against motor axons following parenteral gangliosides. Ann Neurol. 1995;38:218–24.PubMedCrossRefPubMedCentralGoogle Scholar
  32. 32.
    Saleh MN, Khazaeli MB, Wheeler RH, et al. Phase I trial of the murine monoclonal anti-GD2 antibody 14G2a in metastatic melanoma. Cancer Res. 1992;52:4342–7.PubMedPubMedCentralGoogle Scholar
  33. 33.
    Hughes RA, Hadden RD, Gregson NA, Smith KJ. Pathogenesis of Guillain Barre syndrome. J Neuroimmunol. 1999;100:74–97.PubMedCrossRefPubMedCentralGoogle Scholar
  34. 34.
    Kuwabara S, Ogawara K, Misawa S, et al. Does Campylobacter jejuni infection elicit “demyelinating” Guillain-Barré syndrome? Neurology. 2004;63:529–33.PubMedCrossRefPubMedCentralGoogle Scholar
  35. 35.
    Yuki N, Yoshino H, Sato S, et al. Acute axonal polyneuropathy associated with GM1 antibodies following Campylobacter jejuni enteritis. Neurology. 1990;40:1900–3.PubMedCrossRefPubMedCentralGoogle Scholar
  36. 36.
    Koga M, Gilbert M, Li J, et al. Antecedent infections in Fisher syndrome: a common pathogenesis of molecular mimicry. Neurology. 2005;64:1605–11.PubMedCrossRefPubMedCentralGoogle Scholar
  37. 37.
    Hughes RA, Wijdicks EF, Barohn R, Quality Standards Subcommittee of the American Academy of Neurology, et al. Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003;61(6):736–40.PubMedCrossRefPubMedCentralGoogle Scholar
  38. 38.
    The Guillain-Barré Syndrome Study Group. Plasmapheresis and acute Guillain-Barré syndrome. Neurology. 1985;35:1096–104.CrossRefGoogle Scholar
  39. 39.
    French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Plasma exchange in Guillain-Barré syndrome: one-year follow-up. Ann Neurol. 1992;32:94–7.CrossRefGoogle Scholar
  40. 40.
    French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Appropriate number of plasma exchanges in Guillain-Barré syndrome. Ann Neurol. 1997;41:298–306.CrossRefGoogle Scholar
  41. 41.
    Hughes R, Swan A, Raphael J, Annane D, Van Koningsveld R, Van Doorn P. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain. 2007;130:2245–57.PubMedCrossRefPubMedCentralGoogle Scholar
  42. 42.
    Gurses N, Uysal S, Cetinkaya F, Icslek I, Kalayci AG. Intravenous immunoglobulin treatment in children with Guillain-Barré syndrome. Scand J Infect Dis. 1995;27:241–3.PubMedCrossRefPubMedCentralGoogle Scholar
  43. 43.
    Korinthenberg R, Schessl J, Kirschner J, Möntning JS. Intravenous immunoglobulin in the treatment of childhood Guillain-Barré syndrome. Pediatrics. 2005;116:8–14.PubMedCrossRefPubMedCentralGoogle Scholar
  44. 44.
    Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet. 1997;349:225–30.CrossRefGoogle Scholar
  45. 45.
    Nomura T, Hamaguchi K, Hattori T, Satou T, Mannen T, et al. A randomized controlled trial comparing intravenous immunoglobulin and plasmapheresis in Guillain-Barré syndrome. Neurol Ther. 2000;18:69–81.Google Scholar
  46. 46.
    Diener HC, Haupt WF, Kloss TM, Rosenow F, Philipp T, Koeppon S, et al. A preliminary, randomized, multicenter study comparing intravenous immunoglobulin, plasma exchange, and immune adsorption in Guillain-Barré syndrome. Eur Neurol. 2001;46:107–9.PubMedCrossRefPubMedCentralGoogle Scholar
  47. 47.
    Guillain-Barré Syndrome Steroid Trial Group. Double-blind trial of intravenous methylprednisolone in Guillain-Barré syndrome. Lancet. 1993;341:586–90.Google Scholar
  48. 48.
    Garcıa AC, Vidal BE, Rebolledo A, Texeira F, Ordaz FA, Futrán YJ. Treatment of the acute phase of Guillain-Barré-Strohl syndrome with megadosis of methylprednisolone. Rev Invest Clín (Méx). 1985;37:119–24.Google Scholar
  49. 49.
    Shukla SK, Agarwal R, Gupta OP, Pande G, Mamta S. Double blind control trial of prednisolone in Guillain-Barré syndrome – a clinical study. Clinician. 1988;52:128–34.Google Scholar
  50. 50.
    Singh NK, Gupta A. Do corticosteroids influence the disease course or mortality in Guillain-Barré syndrome. J Assoc Physicians India. 1996;44:22–4.PubMedPubMedCentralGoogle Scholar
  51. 51.
    Van Koningsveld R, Schmitz PIM, van der Meché FGA, Dutch GBS Study Group. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet. 2004;363:192–6.PubMedCrossRefPubMedCentralGoogle Scholar
  52. 52.
    Kuwabara S, Masahiro M, Ogawara K, et al. Intravenous immunoglobulin therapy for Guillain-Barre syndrome with IgG anti-GM1 antibody. Muscle Nerve. 2001;24(1):54–8.PubMedCrossRefPubMedCentralGoogle Scholar
  53. 53.
    Ropper AH, Albers JW, Addison R. Limited relapse in Guillain-Barre syndrome after plasma exchange. Arch Neurol. 1988;45(3):314–5.PubMedCrossRefPubMedCentralGoogle Scholar
  54. 54.
    Farcas P, Avnun L, Frisher S, Herishanu YO, Wirguin I. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barré syndrome. Lancet. 1997;350:1747.PubMedCrossRefPubMedCentralGoogle Scholar
  55. 55.
    Bouchard C, Lacroix C, Plante V, et al. Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology. 1999;52:498–503.PubMedCrossRefPubMedCentralGoogle Scholar
  56. 56.
    Rotta FT, Sussman AT, Bradley WG, Ram Ayyar D, Sharma KR, Shebert RT. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 2000;173:129–39.PubMedCrossRefPubMedCentralGoogle Scholar
  57. 57.
    Sakakibara R, Hattori T, Kuwabara S, et al. Micturational disturbance in patients with chronic inflammatory demyelinating polyneuropathy. Neurology. 1998;50:1179–85.PubMedCrossRefPubMedCentralGoogle Scholar
  58. 58.
    Gorson KC, Ropper AH. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): a review of clinical syndromes and treatment approaches in clinical practice. J Clin Neuromuscul Dis. 2003;4(4):174–89.PubMedCrossRefPubMedCentralGoogle Scholar
  59. 59.
    Chio A, Cocito D, Bottacchi E, et al. Idiopathic chronic inflammatory demyelinating polyneuropathy: an epidemiological study in Italy. J Neurol Neurosurg Psychiatry. 2007;78:1349.PubMedPubMedCentralCrossRefGoogle Scholar
  60. 60.
    McCleod 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.CrossRefGoogle Scholar
  61. 61.
    Mygland A, Monstadt P. Chronic polyneuropathies in Vest-Agder, Norway. Eur J Neurol. 2001;8:157–65.PubMedCrossRefPubMedCentralGoogle Scholar
  62. 62.
    Stewart JD, McKelvey R, Durcan L, et al. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetics. J Neurol Sci. 1996;142:59–64.PubMedCrossRefPubMedCentralGoogle Scholar
  63. 63.
    Cocito D, Durelli L, Isoardo G. Different clinical, electrophysiological and immunological features of CIDP associated with paraproteinemia. Acta Neurol Scand. 2003;108:274–80.PubMedCrossRefPubMedCentralGoogle Scholar
  64. 64.
    Simmons Z, Albers JW, Bromberg MB, Feldman EL. Presentation and initial clinical course in patients with chronic inflammatory demyelinating polyradiculoneuropathy: comparison of patients without and with monoclonal gammopathy. Neurology. 1993;43:2202–9.PubMedCrossRefPubMedCentralGoogle Scholar
  65. 65.
    Dyck PJ, Dyck PJB. Atypical varieties of chronic demyelinating neuropathies. Lancet. 2000;355(9212):1293–4.PubMedCrossRefPubMedCentralGoogle Scholar
  66. 66.
    Dyck PJ, Low PA, Windebank AJ, et al. Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N Engl J Med. 1991;325(21):1482–6.PubMedCrossRefPubMedCentralGoogle Scholar
  67. 67.
    Katz JS, Saperstein DS, Gronseth G, Amato AA, Barohn RJ. Distal acquired demyelinating symmetric neuropathy. Neurology. 2000;54(3):615–20.PubMedCrossRefPubMedCentralGoogle Scholar
  68. 68.
    Barohn RJ, Kissel JT, Warmolts JR, Mendell JR. Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol. 1989;46(8):878–84.PubMedCrossRefPubMedCentralGoogle Scholar
  69. 69.
    Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Report from an Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Neurology. 1991;41:617.Google Scholar
  70. 70.
    Dyck PJ, Prineas J, Pollard J. Chronic inflammatory demyelinating polyneuropathy. In: Dyck PJ, Thomas PK, Griffin JW, et al., editors. Peripheral neuropathy. Philadelphia: W.B. Saunders; 1993. p. 1498–517.Google Scholar
  71. 71.
    Krendel DA, Parks HP, Anthony DA, et al. Sural nerve biopsy in chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1989;12:257–64.PubMedCrossRefPubMedCentralGoogle Scholar
  72. 72.
    Kieseier BC, Dalakas MC, Hartung HP. Immune mechanisms in chronic inflammatory demyelinating neuropathy. Neurology. 2002;59:S7.PubMedCrossRefPubMedCentralGoogle Scholar
  73. 73.
    Kiefer R, Kieseier BC, Stoll G, Hartung HP. The role of macrophages in immune-mediated damage to the peripheral nervous system. Prog Neurobiol. 2001;64:109–14.PubMedCrossRefPubMedCentralGoogle Scholar
  74. 74.
    Dalakas MC, Engel WK. Immunoglobulin and complement deposits in nerves of patients with chronic relapsing polyneuropathy. Arch Neurol. 1980;37:637.PubMedCrossRefPubMedCentralGoogle Scholar
  75. 75.
    Dalakas MC, Houff SA, Engel WK, et al. CSF “monoclonal” bands in chronic relapsing polyneuropathy. Neurology. 1980;30:86.CrossRefGoogle Scholar
  76. 76.
    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;47:765–70.PubMedCrossRefPubMedCentralGoogle Scholar
  77. 77.
    Ilyas AA, Mithen FA, Dalakas MC, et al. Antibodies to acidic glycolipids in Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Sci. 1992;107:111–9.PubMedCrossRefPubMedCentralGoogle Scholar
  78. 78.
    Melendez-Vasquez C, Redford J, Choudary PP, et al. Immunological investigation of chronic inflammatory demyelinating polyneuropathy. J Neuroimmunol. 1997;73:124–31.PubMedCrossRefPubMedCentralGoogle Scholar
  79. 79.
    Gabriel CM, Gregson NA, Hughes RA. Anti-PMP22 antibodies in patients with inflammatory neuropathy. J Neuroimmunol. 2000;104:139–45.PubMedCrossRefPubMedCentralGoogle Scholar
  80. 80.
    Ritz MF, Lechner-Scott J, Scott RJ, et al. Characterization of autoantibodies to peripheral myelin protein 22 in patients with hereditary and acquired neuropathies. J Neuroimmunol. 2000;104:155–61.PubMedCrossRefPubMedCentralGoogle Scholar
  81. 81.
    Mathey EK, Garg N, Park SB, Nguyen T, Baker S, Yuki N, Yiannikas C, Kiernan MC, et al. Autoantibody responses to nodal and paranodal antigens in chronic inflammatory neuropathies. J Neuroimmunol. 2017;309:41–6.CrossRefGoogle Scholar
  82. 82.
    Austin JH. Recurrent polyneuropathies and their corticosteroid treatment. With five-year observations of a placebo controlled case treated with corticotrophin, cortisone and prednisone. Brain. 1958;81(2):157.PubMedCrossRefPubMedCentralGoogle Scholar
  83. 83.
    Dyck PJ, O’Brien PC, Oviatt KF, Dinapoli RP, Daube JR, Bartleson JD, et al. Prednisone improves chronic inflammatory polyradiculoneuropathy more than no treatment. Ann Neurol. 1982;11:136–41.PubMedCrossRefPubMedCentralGoogle Scholar
  84. 84.
    Mehndiratta MM, Hughes RA. Corticosteroids for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2002;11:CD002062.Google Scholar
  85. 85.
    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.PubMedCrossRefPubMedCentralGoogle Scholar
  86. 86.
    Hahn AF, Bolton CF, Pillay N, Chalk C, Benstead T, Bril V, et al. Plasma exchange in chronic inflammatory demyelinating polyradiculoneuropathy. A double blind, sham-controlled, crossover study. Brain. 1996;119(Pt 4):1055–66.PubMedCrossRefPubMedCentralGoogle Scholar
  87. 87.
    Eftimov F, Winer JB, Vermeulen M, de Haan R, van Schaik IN. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev. 2009;(1):CD001797.
  88. 88.
    Hughes RA, Donofrio P, Bril V, et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 2008;7(2):136–44.PubMedCrossRefPubMedCentralGoogle Scholar
  89. 89.
    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(2):195–201.PubMedCrossRefPubMedCentralGoogle Scholar
  90. 90.
    Dyck PJ, Litchy WJ, Kratz KM, Suarez GA, Low PA, Pineda AA, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36(6):838–45.PubMedCrossRefPubMedCentralGoogle Scholar
  91. 91.
    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.PubMedCrossRefPubMedCentralGoogle Scholar
  92. 92.
    Dalakas MC, Engel WK. Chronic relapsing (dysimmune) polyneuropathy: pathogenesis and treatment. Ann Neurol. 1981;9(Suppl):134–45.PubMedCrossRefPubMedCentralGoogle Scholar
  93. 93.
    Brannagan TH, Pradhan A, Heiman-Patterson T, Winkelman AC, Styler MJ, Topolsky DL, et al. High-dose cyclophosphamide without stem-cell rescue for refractory CIDP. Neurology. 2002;58(12):1856–8.PubMedCrossRefPubMedCentralGoogle Scholar
  94. 94.
    McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain. 1987;110(Pt 6):1617–30.PubMedCrossRefPubMedCentralGoogle Scholar
  95. 95.
    Gladstone DE, Prestrud AA, Brannagan TH III. 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.PubMedCrossRefPubMedCentralGoogle Scholar
  96. 96.
    Good JL, Chehrenama M, Mayer RF, Koski CL. Pulse cyclophosphamide therapy in chronic inflammatory demyelinating polyneuropathy. Neurology. 1998;51(6):1735–8.PubMedCrossRefPubMedCentralGoogle Scholar
  97. 97.
    Barnett MH, Pollard JD, Davies L, McLeod JG. Cyclosporin A in resistant chronic inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1998;21(4):454–60.PubMedCrossRefPubMedCentralGoogle Scholar
  98. 98.
    Hodgkinson SJ, Pollard JD, McLeod JG. Cyclosporin A in the treatment of chronic demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry. 1990;53(4):327–30.PubMedPubMedCentralCrossRefGoogle Scholar
  99. 99.
    Mahattanakul W, Crawford TO, Griffin JW, Goldstein JM, Cornblath DR. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A. J Neurol Neurosurg Psychiatry. 1996;60(2):185–7.PubMedPubMedCentralCrossRefGoogle Scholar
  100. 100.
    Matsuda M, Hoshi K, Gono T, Morita H, Ikeda S. Cyclosporin A in treatment of refractory patients with chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 2004;224(1–2):29–35.PubMedCrossRefPubMedCentralGoogle Scholar
  101. 101.
    Odaka M, Tatsumoto M, Susuki K, Hirata K, Yuki N. Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with cyclosporin. J Neurol Neurosurg Psychiatry. 2005;76(8):1115–20.PubMedPubMedCentralCrossRefGoogle Scholar
  102. 102.
    Dyck PJ, O’Brien P, Swanson C, Low P, Daube J. Combined azathioprine and prednisone in chronic inflammatory demyelinating polyneuropathy. Neurology. 1985;35(8):1173–6.PubMedCrossRefPubMedCentralGoogle Scholar
  103. 103.
    Vallat JM, Hahn AF, Léger JM, Cros DP, Magy L, Tabaraud F, et al. Interferon beta-1a as an investigational treatment for CIDP. Neurology. 2003;60(8 Suppl 3):23–8.CrossRefGoogle Scholar
  104. 104.
    Hughes RAC, Gorson KC, Cros D, Griffin J, Pollard J, Vallat J-M, Maurer SL, Riester K, Davar G, Dawson K, Sandrock A, Avonex® CIDP Study Group. Intramuscular interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 2010;74:651–7.PubMedCrossRefPubMedCentralGoogle Scholar
  105. 105.
    Querol L, Rojas-García R, Diaz-Manera J, Barcena J, Pardo J, Ortega-Moreno A, Sedano MJ, Illa I, et al. Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins. Neurol Neuroimmunol Neuroinflamm. 2015;2(5):e149.PubMedPubMedCentralCrossRefGoogle Scholar
  106. 106.
    Marsh E, Hirst C, Llewelyn J, Cossburn M, et al. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy. J Neurol. 2010;257(6):913–9.PubMedCrossRefPubMedCentralGoogle Scholar
  107. 107.
    Gorson K, Ropper A, Clark B, et al. Treatment of chronic inflammatory demyelinating polyneuropathy with interferon-alpha 2a. Neurology. 1998;50(1):84–7.PubMedCrossRefPubMedCentralGoogle Scholar
  108. 108.
    Hadden R, Sharrack B, Bensa S, et al. Randomized trial of interferon beta-1a in chronic inflammatory demyelinating polyradiculoneuropathy. Neurology. 1999;53(1):57–61.PubMedCrossRefPubMedCentralGoogle Scholar
  109. 109.
    RMC Trial Group. Randomised controlled trial of methotrexate for chronic inflammatory demyelinating polyradiculoneuropathy (RMC Trial): a pilot, multicentre study. Neurology. 2009;8(2):158–64.Google Scholar
  110. 110.
    Mahdi-Rogers M, Kazmi M, Ferner R, et al. Autologous peripheral blood stem cell transplantation for chronic acquired demyelinating neuropathy. J Peripher Nerv Syst. 2009;14(2):118–24.PubMedCrossRefPubMedCentralGoogle Scholar
  111. 111.
    Lewis R, Sumner A, Brown MJ, Asbury AK. Chronic multifocal demyelinative neuropathy: a unique disorder with persistent conduction block. Neurology. 1982;32:958–62.PubMedCrossRefPubMedCentralGoogle Scholar
  112. 112.
    Viala K, Renie L, Maisonobe T, et al. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain. 2004;127:2010–7.PubMedCrossRefPubMedCentralGoogle Scholar
  113. 113.
    Oh SJ, Joy JJ, Kuruoglu R. “Chronic sensory demyelinating neuropathy”: chronic inflammatory demyelinating polyneruopathy presenting as a pure sensory neuropathy. J Neurol Neurosurg Psychiatry. 1992;55:677.PubMedPubMedCentralCrossRefGoogle Scholar
  114. 114.
    Chin RL, Latov N, Sander HW, et al. Sensory CIDP presenting as cryptogenic sensory polyneuropathy. J Peripher Nerv Syst. 2004;9:132–7.PubMedCrossRefPubMedCentralGoogle Scholar
  115. 115.
    Berger AR, Herskovitz S, Kaplan J. Late motor involvement in cases presenting as “chronic demyelinating polyneuropathy”. Muscle Nerve. 1995;18(4):440–4.PubMedCrossRefPubMedCentralGoogle Scholar
  116. 116.
    Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve. 1988;11:103–7.PubMedCrossRefPubMedCentralGoogle Scholar
  117. 117.
    Lewis R. Conduction block neuropathies. Curr Opin Neurol. 2007;20(5):525–30.PubMedCrossRefPubMedCentralGoogle Scholar
  118. 118.
    Nobile-Orazio E. Multifocal motor neuropathy. J Neuroimmunol. 2001;115(1–2):4–18.PubMedCrossRefPubMedCentralGoogle Scholar
  119. 119.
    Kornberg AJ, Pestronk A. The clinical and diagnostic role of anti-GM1 antibody testing. Muscle Nerve. 1994;17:100–4.PubMedCrossRefPubMedCentralGoogle Scholar
  120. 120.
    Taylor BV, Gross LA, Windebank AJ. The sensitivity and specificity of anti-GM1 antibody testing. Neurology. 1996;47:951–5.PubMedCrossRefPubMedCentralGoogle Scholar
  121. 121.
    Corse AM, Chaudry W, Crawford TO, Cornblath DR, Kuncl RW, Griffin JW. Sural nerve pathology in multifocal motor neuropathy. Ann Neurol. 1996;39:319–25. Comment in: Ann Neurol. 1996;40:948–50.PubMedCrossRefPubMedCentralGoogle Scholar
  122. 122.
    Taylor BV, Dyck PJB, Engelstand J, Gruener G, Grant I, Dyck PJ. Multifocal motor neuropathy: pathologic alterations at the site of conduction block. J Neuropathol Exp Neurol. 2004;63:129–37.PubMedCrossRefPubMedCentralGoogle Scholar
  123. 123.
    Nobile-Orazio E, Cappellari A, Priori A. Multifocal motor neuropathy: current concepts and controversies. Muscle Nerve. 2005;31:663–80.PubMedCrossRefPubMedCentralGoogle Scholar
  124. 124.
    Santoro M, Uncini A, Corbo M, Staugaitis SM, Thomas FP, Hays AP, et al. Experimental conduction block induced by serum from a patient with anti-GM1 antibodies. Ann Neurol. 1992;31:385–90.PubMedCrossRefPubMedCentralGoogle Scholar
  125. 125.
    Arasaki K, Kusunoki S, Kudo N, Kanazawa I. Acute conduction block in vitro following exposure to anti-ganglioside sera. Muscle Nerve. 1993;16:587–93.PubMedCrossRefPubMedCentralGoogle Scholar
  126. 126.
    Harvey GK, Toyka KV, Zielasek J, Kiefer R, Simonis C, Hartung HP. Failure of anti-GM1 IgG or IgM to induce conduction block following intraneural transfer. Muscle Nerve. 1995;18:388–94.PubMedCrossRefPubMedCentralGoogle Scholar
  127. 127.
    Sugie K, Murataq K, Ikoma K, et al. A case of acute multifocal motor neuropathy with conduction block after Campylobacter jejuni enteritis. Rinsho Shinkeigaku. 1998;38:436.Google Scholar
  128. 128.
    Pestronk A, Cornblath DR, Ilyas A, Baba H, Quarles RH, Griffin JW, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988;24:73–8.PubMedCrossRefPubMedCentralGoogle Scholar
  129. 129.
    Azulay JP, Blin O, Pouget J, Boucraut J, Billé-Turc F, Carles G, et al. Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study. Neurology. 1994;44(3 Pt 1):429–32.PubMedCrossRefPubMedCentralGoogle Scholar
  130. 130.
    Federico P, Zochodne DW, Hahn AF, Brown WF, Feasby TE. Multifocal motor neuropathy improved by IVIg: randomized, double-blind, placebo-controlled study. Neurology. 2000;55(9):1256–62.PubMedCrossRefPubMedCentralGoogle Scholar
  131. 131.
    Léger J-M, Chassande B, Musset L, Meininger V, Bouche P, Baumann N. Intravenous immunoglobulin therapy in multifocal motor neuropathy: a double-blind, placebo-controlled study. Brain. 2001;124(1):145–53.PubMedCrossRefPubMedCentralGoogle Scholar
  132. 132.
    Van den Berg LH, Kerkhoff H, Oey PL, Franssen H, Mollee I, Vermeulen M, et al. Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double blind, placebo controlled study. J Neurol Neurosurg Psychiatry. 1995;59(3):248–52.PubMedPubMedCentralCrossRefGoogle Scholar
  133. 133.
    Pestronk A, Chaudhry V, Feldman EL, Griffin JW, Cornblath DR, Denys EH, et al. Lower motor neuron syndromes defined by patterns of weakness, nerve conduction abnormalities, and high titers of anti-glycolipid antibodies. Ann Neurol. 1990;27(3):316–26.PubMedCrossRefPubMedCentralGoogle Scholar
  134. 134.
    Feldman EL, Bromberg MB, Albers JW, Pestronk A. Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol. 1991;30(3):397–401.PubMedCrossRefPubMedCentralGoogle Scholar
  135. 135.
    Piepers S, Van den Berg-Vos R, Van der Pol WL, Franssen H, Wokke J, Van den Berg L. Mycophenolate mofetil as adjunctive therapy for MMN patients: a randomized, controlled trial. Brain. 2007;130(Pt 8):2004–10.PubMedCrossRefPubMedCentralGoogle Scholar
  136. 136.
    Kelly JJ, Kyle RA, O’Brien PC, Dyck PJ. Prevalence of monoclonal protein in peripheral neuropathy. Neurology. 1981;31(11):1480–3.PubMedCrossRefPubMedCentralGoogle Scholar
  137. 137.
    Ropper AH, Gorson KC. Neuropathies associated with paraproteinemia. N Engl J Med. 1998;338(22):1601–7.PubMedCrossRefPubMedCentralGoogle Scholar
  138. 138.
    Levine T, Pestronk A, Florence J, et al. Peripheral neuropathies in Waldenström’s macroglobulinaemia. J Neurol Neurosurg Psychiatry. 2006;77:224–8. This report describes the symptoms and signs of peripheral neuropathy in a cohort of patients with Waldenström’s macroglobulinaemia.PubMedPubMedCentralCrossRefGoogle Scholar
  139. 139.
    Noronha V, Fynan TM, Duffy T. Flare in neuropathy following rituximab therapy in Waldenstrom’s macroglobulinemia. J Clin Oncol. 2006;24(1):e3.PubMedCrossRefPubMedCentralGoogle Scholar
  140. 140.
    Mauermann ML, Ryan ML, Moon JS, Klein CJ. Case of mononeuropathy multiplex onset with rituximab therapy for Waldenstrom’s macroglobulinemia. J Neurol Sci. 2007;260:240–3.PubMedCrossRefPubMedCentralGoogle Scholar
  141. 141.
    Kelly JJ Jr, Kyle RA, Miles JM, O’Brien PC, Dyck PJ. The spectrum of peripheral neuropathy in myeloma. Neurology. 1981;31:24–31.PubMedCrossRefPubMedCentralGoogle Scholar
  142. 142.
    Drieger H, Pruzanski W. Plasma cell neoplasia with peripheral polyneuropathy. Medicine. 1988;59:301–10.CrossRefGoogle Scholar
  143. 143.
    Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve. 1998;21:1390–7.PubMedCrossRefPubMedCentralGoogle Scholar
  144. 144.
    Gosselin S, Kyle RA, Dyck PJ. Neuropathy associated with monoclonal gammopathies of undetermined significance. Ann Neurol. 1991;30:54–61.PubMedCrossRefPubMedCentralGoogle Scholar
  145. 145.
    Yeung KB, Thomas PK, King RHM, et al. The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraproteinemia: comparative clinical, immunological and nerve biopsy findings. J Neurol. 1991;238:383–91.PubMedCrossRefPubMedCentralGoogle Scholar
  146. 146.
    Latov N, Hays AP, Sherman WH. Peripheral neuropathy and anti-MAG antibodies. Crit Rev Neurobiol. 1988;3:301–32.PubMedPubMedCentralGoogle Scholar
  147. 147.
    Kissel JT, Collins MP. Peripheral nerve vasculitis. In: Younger DS, editor. Motor disorders. Philadelphia: Lippincott Williams and Wilkins; 1999. p. 243–56.Google Scholar
  148. 148.
    Hughes RAC. Guillain-Barré syndrome. Heidelberg: Springer-Verlag; 1998.Google Scholar
  149. 149.
    Griffin JW, Li CY, Macko C, et al. Early nodal changes in the acute motor axonal neuropathy pattern of the Guillain-Barré syndrome. J Neurocytol. 1996;25:33–51.PubMedCrossRefPubMedCentralGoogle Scholar
  150. 150.
    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.PubMedCrossRefPubMedCentralGoogle Scholar
  151. 151.
    Pytel P, Rezania K, Soliven B, Frank J, Wollmann R. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with hypertrophic spinal radiculopathy mimicking neurofibromatosis. Acta Neuropathol. 2003;105:185–8.PubMedPubMedCentralGoogle Scholar
  152. 152.
    Van Den Berg-Vos RM, Van Den Berg LH, Visser J, de Visser M, Franssen H, Wokke JHJ. The spectrum of lower motor neuron disorders. J Neurol. 2003;250:1279–92.CrossRefGoogle Scholar
  153. 153.
    Vital A, Lagueny A, Julien J, et al. Chronic inflammatory demyelinating polyneuropathy with dysglobulinemia: a peripheral nerve biopsy study in 18 cases. Acta Neuropathol. 2000;100:63–8.PubMedCrossRefPubMedCentralGoogle Scholar

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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of NeurologyMidcoast HospitalBrunswickUSA
  2. 2.Department of NeurologyWarren Alpert Medical School of Brown University, Rhode Island HospitalProvidenceUSA

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