Treatment of Inflammatory Demyelinating Polyneuropathy

  • G. Comi
  • L. Roveri
Part of the Topics in Neuroscience book series (TOPNEURO)


The management of acute and chronic inflammatory demyelinating polyneuropathy (Guillain-Barrè syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)) will be the main topic of this chapter. A few comments will also be made about treatment of the demyelinating form of paraproteinaemic demyelinating polyneuropathy (PDN) and of multifocal motor neuropathy (MMN). We will briefly describe the main characteristics of these neuropaties and examines case series and trials which evaluated the principal therapeutic strategies for GBS, CIDP, PDN and MMN, such as intravenous immunoglobulin therapy (IVIg), steroid treatment, plasma exchange, and immunosuppressor administration. Controlled trials demonstrated that IVIg, steroid treatment and plasma exchange are effective in GBS and CIDP. For PDN, the therapeutic strategies are the same as for idiopathic CIDP, but usually the clinical response is poorer. For MMN, IVIg are definitely the first choice treatment.


Plasma Exchange Neurol Neurosurg Psychiatry Monoclonal Gammopathy Chronic Inflammatory Demyelinating Polyneuropathy Multifocal Motor Neuropathy 


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  1. 1.
    Winer JB, Hughes RAC, Osmond C (1988) A prospective study of acute idiopathic neuropathy. I. Clinical features and their prognostic value. J Neurol Neurosurg Psychiatry 51: 605–612PubMedCrossRefGoogle Scholar
  2. 2.
    Alters M (1990) The epidemiology of Guillain-Barrè syndrome. Ann Neurol 27(Suppl): S7–12CrossRefGoogle Scholar
  3. 3.
    Arnason BGW, Soliven B (1993) Acute inflammatory demyelinating polyradiculoneuropathy. In: Dyck PJ, Thomas PK, Griffin JW et al. (eds) Peripheral neuropathy, 3rd edn. WB Saunders, Philadelphia, pp 1437–1497Google Scholar
  4. 4.
    Ng KKP, Howard RS, Fish DR et al. (1995) Management and outcome of severe Guillain-Barrè syndrome. QJM 88: 243–250 (abstract)PubMedGoogle Scholar
  5. 5.
    Bos AP, van der Mechè FGA, Witsenburg M, van der Voort E (1987) Experiences with Guillain-Barrè syndrome in a paediatric intensive care unit. Intens Care Med 13: 328–331CrossRefGoogle Scholar
  6. 6.
    Rees JH, Thompson RD, Hughes RAC (1996) An epidemiological study of Guillain-Barrè syndrome. J Neurol Neurosurg Psychiatry 61: 215 (abstract)Google Scholar
  7. 7.
    Rees JH, Gregson NA, Griffiths PL et al. (1993) Campylobacter jejuni and Guillain-Barrè syndrome. QJM 86: 623–624PubMedCrossRefGoogle Scholar
  8. 8.
    Mishu B, Ilya AA, Koski CL et al. (1993) Serologic evidence of previous Campylobacter jejuni infection in patients with the Guillain-Barrè syndrome. Ann Intern Med 118: 947–953PubMedGoogle Scholar
  9. 9.
    Rees JH, Soudain SE, Gregson NA, Hughes RAC (1995) Campylobacter jejuni infection and Guillain-Barrè syndrome. N Engl J Med 333: 1374–1379PubMedCrossRefGoogle Scholar
  10. 10.
    Jacobs BC, van Doorn PA, Schmitz PIM et al. (1996) Campylobacter jejuni infection and anti-GM1 antibodies in Guillain-Barrè syndrome. Ann Neurol 40: 181–187PubMedCrossRefGoogle Scholar
  11. 11.
    Steele JC, Gladstone RM, Thanasophon S et al. (1969) Mycoplasma pneumoniae as a determinant of the Guillain-Barrè syndrome. Lancet 2(7623): 710–713PubMedGoogle Scholar
  12. 12.
    Dowling PC, Cook SB (1981) Role of infection in Guillain-Barrè syndrome: laboratory confirmation of herpes virus in 41 cases. Ann Neurol 9(suppl): 44–45PubMedCrossRefGoogle Scholar
  13. 13.
    Winer JB, Hughes RAC, Anderson MJ et al. (1988) A prospective study of acute idiopathic neuropathy: II antecedent events. J Neurol Neurosurg Psychiatry 11: 613–618CrossRefGoogle Scholar
  14. 14.
    Boucquey D, Sindic CJM, Lamy M et al. (1991) Clinical and serological studies in a series of 45 patients with Guillain-Barrè syndrome. J Neurol Sci 104: 56–63PubMedCrossRefGoogle Scholar
  15. 15.
    Visser LH, van der Mechè FGA, Meulstee J et al. (1996) Cytomegalovirus infection and Guillain-Barrè syndrome: the clinical, electrophysiologic, and prognostic features. Neurology 47: 668–673PubMedCrossRefGoogle Scholar
  16. 16.
    Gautier-Smith PC (1965) Neurological complication of glandular fever (infectious mononucleosis). Brain 88: 323–334PubMedCrossRefGoogle Scholar
  17. 17.
    Berger JR, Difini JA, Swerdloff MA et al. (1987) HIV seropositivity in Guillain-Barrè syndrome. Ann Neurol 22: 393–394PubMedCrossRefGoogle Scholar
  18. 18.
    Gross FJ, Mindel JS (1991) Pseudotumor cerebri and Guillain-Barrè syndrome associated with human immunodeficiency virus infection. Neurology 41: 1845–1846PubMedGoogle Scholar
  19. 19.
    Lacaille F, Zylberberg H, Hagegè H et al. (1998) Hepatitis C associated with Guillain-Barrè syndrome. Liver 18: 49–51PubMedGoogle Scholar
  20. 20.
    Kaplan JE, Schonberger LB, Hurwitz ES, Katona P (1983) Guillain-Barrè syndrome in the United States, 1978–1981: additional observation from the national surveillance system. Neurology 33: 633–637PubMedCrossRefGoogle Scholar
  21. 21.
    Blaser MJ (1997) Epidemiologic and clinical features of Campylobacter jejuni infections. J Infect Dis 176(suppl 2): S103–105PubMedCrossRefGoogle Scholar
  22. 22.
    Roscelli JD, Bass JW, Pang L (1991) Guillain-Barrè syndrome and influenza vaccination in the US Army, 1980–1988. Am J Epidemiol 133: 952–955PubMedGoogle Scholar
  23. 23.
    Hughes RAC, Rees J, Smeeton N, Winer J (1996) Vaccines and Guillain-Barrè syndrome. BMJ 312: 1475–1476PubMedGoogle Scholar
  24. 24.
    De Silveira CM, Salisbury DM, De Quadros CA (1997) Measles vaccination and Guillain-Barrè syndrome. Lancet 349: 14–15PubMedCrossRefGoogle Scholar
  25. 25.
    Kinnunen E, Junttila O, Haukka J, Hovi T (1998) Nationwide oral poliovirus vaccination campaign and the incidence of Guillain-Barrè syndrome. Am J Epidemiol 147: 69–73PubMedCrossRefGoogle Scholar
  26. 26.
    Salisbury DM (1998) Association between oral poliovaccine and Guillain-Barrè syndrome? Lancet 351: 79–80PubMedCrossRefGoogle Scholar
  27. 27.
    Hemachudha T, Griffin DE, Chen WW, Johnson RT (1988) Immunologic studies of rabies vaccination-induced Guillain-Barrè syndrome. Neurology 38: 375–278PubMedCrossRefGoogle Scholar
  28. 28.
    Knittel TH, Ramadori G, Majet WJ et al. (1989) Guillain-Barrè syndrome and human diploid cell rabies vaccine. Lancet 1(8649): 1334–1335Google Scholar
  29. 29.
    Arrowsmith JB, Milstain JB, Kuritsky JN et al. (1985) Streptokinase and Guillain-Barrè syndrome. Ann Intern Med 103: 302 (letter)Google Scholar
  30. 30.
    Chakraborty TK, Ruddell WS (1987) Guillain-Barrè neuropathy during treatment with captopril. Postgrad Med J 63: 221–222PubMedCrossRefGoogle Scholar
  31. 31.
    Hory B, Blanc D, Boillot A et al. (1985) Guillain-Barrè syndrome following danazol and corticosteriod therapy for hereditary angioedema. Am J Med 79: 111–115PubMedCrossRefGoogle Scholar
  32. 32.
    Loizou LA, Boddie HG (1978) Polyradiculoneuropathy associated with heroin abuse. J Neurol Neurosurg Psychiatry 41: 855–857PubMedCrossRefGoogle Scholar
  33. 33.
    Giovannoni G, Hartung H-P (1996) The immunopathogenesis of multiple sclerosis and Guillain-Barrè syndrome. Curr Opin Neurol 9: 165–177PubMedCrossRefGoogle Scholar
  34. 34.
    Hartung H-P, Pollard JD, Harvey GK, Toyka KV (1995) Immunopathogenesis and treatment of the Guillain-Barrè syndrome-Part I. Muscle Nerve 18: 137–153PubMedCrossRefGoogle Scholar
  35. 35.
    Hartung H-P, Pollard JD, Harvey GK, Toyka KV (1995) Immunopathogenesis and treatment of the Guillain-Barrè syndrome-Part II. Muscle Nerve 18: 154–164PubMedCrossRefGoogle Scholar
  36. 36.
    Honavar M, Tharakan JHJ, Hughes RAC et al. (1991) A clinico-pathological study of the Guillain-Barrè syndrome: nine cases and literature review. Brain 114: 1245–1269PubMedCrossRefGoogle Scholar
  37. 37.
    Griffin JW, Li CY, Ho TW et al. (1995) Guillain-Barrè syndrome in northern China: the spectrum of neuropathological changes in clinically defined cases. Brain 118: 577–595PubMedCrossRefGoogle Scholar
  38. 38.
    Guillain G, Barrè JA, Strohl A (1916) Sur un syndrome de radiculonevritè avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire: remarques surles caractères cliniques et graphiques des réflexes tendineux. Bull Soc Med Hop Paris 40: 1462–1470Google Scholar
  39. 39.
    Feasby TE, Gilbert JJ, Brown WF et al. (1986) An acute axonal form of Guillain-Barrè polyneuropathy. Brain 109: 1115–1116PubMedCrossRefGoogle Scholar
  40. 40.
    Yuki N, Yoshino H, Sato S et al. (1992) Severe acute axonal form of Guillain-Barrè syndrome associated with IgG anti-GD1a antibodies. Muscle Nerve 15: 899–903PubMedCrossRefGoogle Scholar
  41. 41.
    Brown WF, Feasby TE, Hahn AF (1993) Electrophysiological changes in the acute “axonal” form of Guillain-Barrè syndrome. Muscle Nerve 16: 200–205PubMedCrossRefGoogle Scholar
  42. 42.
    Hafer-Macko C, Hsieh S-T, Li CY et al. (1996) Acute motor axonal neuropathy: an antibody-mediated attack on axolemma. Ann Neurol 40: 635–644PubMedCrossRefGoogle Scholar
  43. 43.
    Griffin JW, Li CY, Macko C et al. (1996) Early nodal changes in the acute motor axonal neuropathy pattern of the Guillain-Barrè syndrome. J Neurocytol 25: 33–51PubMedCrossRefGoogle Scholar
  44. 44.
    Chiba A, Kusonoki S, Shimizu T, Kanazawa I (1992) Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller-Fisher syndrome. Ann Neurol 31: 677–679PubMedCrossRefGoogle Scholar
  45. 45.
    Willison HJ, Veitch J, Paterson G, Kennedy PGE (1993) Miller-Fisher syndrome is associated with serum antibodies to GB1b ganglioside. J Neurol Neurosurg Psychiatry 56: 204–206PubMedCrossRefGoogle Scholar
  46. 46.
    Chiba A, Kusonoki S, Obata H et al. (1993) Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller-Fisher syndrome and Guillain-Barrè syndrome: clinical and immunohistochemical studies. Neurology 43: 1911–1917PubMedCrossRefGoogle Scholar
  47. 47.
    Kornberg AJ, Pestronk A, Blume GM et al. (1996) Selective staining of the cerebellar molecular layer by serum IgG in Miller-Fisher and related syndrome. Neurology 47: 1317–1320PubMedCrossRefGoogle Scholar
  48. 48.
    McKann GM, Griffin JW, Cornblath DR et al. (1988) Plasmapheresis and Guillain-Barrè syndrome: analysis of prognostic factors and the effect of plasmapheresis. Ann Neurol 23: 347–353CrossRefGoogle Scholar
  49. 49.
    French Cooperative Group on Plasma Exchange in Guillain-Barrè Syndrome (1987) Efficiency of plasma exchange in Guillain-Barrè syndrome: role of replacement fluids. Ann Neurol 22: 753–761CrossRefGoogle Scholar
  50. 50.
    French Cooperative Group on Plasma Exchange in Guillain-Barrè Syndrome (1992) Plasma exchange in Guillain-Barrè syndrome: one-year follow-up. Ann Neurol 32: 94–97CrossRefGoogle Scholar
  51. 51.
    Kleyweg RP, van der Meché FGA (1991) Treatment related fluctuation in Guillain-Barrè syndrome after high-dose immunoglobulins or plasma exchange. J Neurol Neurosurg Psychiatry 54: 957–960PubMedCrossRefGoogle Scholar
  52. 52.
    Rudnicki S, Vriesendorp F, Koski CI, Mayer RF (1992) Electrophysiologic studies in the Guillain-Barrè syndrome: effects of plasma exchange and antibody rebound. Muscle Nerve 15: 57–62PubMedCrossRefGoogle Scholar
  53. 53.
    French Cooperative Group on Plasma Exchange in Guillain-Barrè Syndrome (1997) Appropriate numbers of plasma exchanges in the Guillain-Barrè syndrome. Ann Neurol 41: 298–306CrossRefGoogle Scholar
  54. 54.
    Vermeulen M, van der Meché FGA, Speelman JD (1985) Plasma exchange and gamma-globulin infusion in chronic inflammatory polyneuropathy. J Neurol Sci 70: 317–326PubMedCrossRefGoogle Scholar
  55. 55.
    Curro Dossi B, Tezzon F (1987) High-dose intravenous gammaglobunin for chronic inflammatory demyelinating polyneuropathy. Ital J Neurol Sci 8: 321–326PubMedCrossRefGoogle Scholar
  56. 56.
    van der Meché FGA, Schmitz PIM, Dutch Guillain-Barrè Syndrome Study Group (1992) A randomised trial comparing intravenous immunoglobulin and plasma exchange in Guillain-Barrè syndrome. N Engl J Med 326: 1123–1129PubMedCrossRefGoogle Scholar
  57. 57.
    Plasma Exchange/Sandoglobulin Guillain-Barrè Syndrome Trial Group (1997) Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barrè syndrome. Lancet 349: 225–230CrossRefGoogle Scholar
  58. 58.
    Epstein MA, Sladky JT (1990) The role of plasmapheresis in childhood Guillain-Barrè syndrome. Ann Neurol 28: 65–69PubMedCrossRefGoogle Scholar
  59. 59.
    Shahar E, Murphy EG, Roifman CM (1990) Benefit of intravenously administered immune serum globulin in patients with Guillain-Barrè syndrome. J Pediatric 116: 141–144CrossRefGoogle Scholar
  60. 60.
    Vajsar J, Sloane A, Wood E et al. (1994) Plasmapheresis versus intravenous immunoglobulin treatment in childhood Guillain-Barrè syndrome. Arch Pediatr Adolesc Med 148: 1210–1212PubMedCrossRefGoogle Scholar
  61. 61.
    Irani DN, Cornblath DR, Chaudhry V et al. (1993) Relapse in Guillain-Barrè syndrome after treatment with human immune globulin. Neurology 43: 872–875PubMedCrossRefGoogle Scholar
  62. 62.
    Castro LH, Ropper AH (1993) Human immune globulin infusion in Guillain-Barrè syndrome: worsening during and after treatment. Neurology 43: 1034–1036PubMedCrossRefGoogle Scholar
  63. 63.
    Romano JG, Rotta FT, Potter P et al. (1998) Relapses in the Guillain-Barrè syndrome after treatment with intravenous immune globulin or plasma exchange. Muscle Nerve 21: 1327–1330PubMedCrossRefGoogle Scholar
  64. 64.
    Visser LH, van der Meché FGA, Meulstee J et al. (1998) Risk factors for treatment related clinical fluctuations in Guillain-Barrè syndrome. J Neurol Neurosurg Psychiatry 64: 242–244PubMedCrossRefGoogle Scholar
  65. 65.
    Guillain-Barrè Syndrome Steroid Trial Group (1993) Double-blind trial of intravenous methylprednisolone in Guillain-Barrè syndrome. Lancet 341: 586–589Google Scholar
  66. 66.
    Dutch Guillain-Barrè Study Group (1994) Treatment of Guillain-Barrè syndrome with high-dose methylprednisolone: a pilot study. Ann Neurol 35: 749–752CrossRefGoogle Scholar
  67. 67.
    Choudhary PP, Thompson N, Hughes RAC (1995) Improvement following interferon beta in chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol 242: 252–253PubMedCrossRefGoogle Scholar
  68. 68.
    Hughes RAC (1998) Modulating the immune response in demyelinating diseases. J Neurol Neurosurg Psychiatry 64(2): 148PubMedCrossRefGoogle Scholar
  69. 69.
    Créange A, Lerat H, Meyrignac C, Degos J-D et al. (1998) Treatment of Guillain-Barrè syndrome with interferon-β. Lancet 352: 368–369PubMedCrossRefGoogle Scholar
  70. 70.
    Hartung H-P, Reiners K, Toyka KV, Pollard JD (1994) Guillain-Barrè syndrome and CIDP. In: Hohlfeld R (ed) Immunology of neuromuscular disease. Kluwer, Dordrecht, pp 33–104CrossRefGoogle Scholar
  71. 71.
    Prineas JW, McLeod JG (1976) Chronic relapsing polyneuritis. J Neurol Sci 27: 427–458PubMedCrossRefGoogle Scholar
  72. 72.
    Koski CL, Humphrey R, Shin ML (1985) Anti-peripheral myelin antibody in patients with demyelinating neuropathy: quantitative and kinetic determination of serum antibody by complement component 1 fixation. Proc Natl Acad Sci USA 82: 905–909PubMedCrossRefGoogle Scholar
  73. 73.
    van Doorn PA, Brand A, Vermeulen M (1987) Clinical significance of antibodies against peripheral nerve tissue in inflammatory polineuropathy. Neurology 37: 1798–1802PubMedCrossRefGoogle Scholar
  74. 74.
    Fredman P, Vedeler CA, Nyland H ET AL. (1991) Antibodies in antisera from patients with inflammatory demyelinating poliradiculo-neuropathy react with ganglioside LM1 and sulfatide of periferal nerve myelin. J Neurol 238: 76–79CrossRefGoogle Scholar
  75. 75.
    Conolly AM, Pestronk A, Trotter JL et al. (1993) High titer selective serum anti tubulin antibodies in chronic inflammatory demyelinating polyneuropathy. Neurology 43: 557–562CrossRefGoogle Scholar
  76. 76.
    Simone IL, Annunziata P, Maimone D (1993) Serum and CSF antibodies to ganglioside GM1 in Guillain-Barrè syndrome and chronic inflammatory polyneuropathy. J Neurol Sci 114: 49–55PubMedCrossRefGoogle Scholar
  77. 77.
    Saida K, Sumner AJ, Saida T (1980) Antiserum mediated demyelinization: relationship between remyelinization and functional recovery. Ann Neurol 8: 12–24PubMedCrossRefGoogle Scholar
  78. 78.
    Saida T, Saida K, Lisak RP et al. (1982) In vivo demyelinating activity of sera from patients with Guillain-Barrè syndrome. Ann Neurol 11: 69–75PubMedCrossRefGoogle Scholar
  79. 79.
    Heininger K, Liebert UG, Toyka KV, Haneveld FT et al. (1984) Chronic inflammatory demyelinating polyradiculoneuropathy. Reduction of nerve conduction velocities in monkeys by systemic passive transfer of immunoglobulin G. J Neurol Sci 66: 1–14PubMedCrossRefGoogle Scholar
  80. 80.
    Lassmann H, Fierz W, Neuchrist C et al. (1991) Chronic relapsing esperimental allergic neuritis induced by repeated transfer of P2-protein reactive T cell lines. Brain 144: 429–442CrossRefGoogle Scholar
  81. 81.
    van Doorn PA, Vermeulen M, Brand A et al. (1991) Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy. Clinical and laboratory characteristics associated with improvement. Arch Neurol 48: 217–220PubMedCrossRefGoogle Scholar
  82. 82.
    McCombe PA, Pollard JD, McLeod JG (1987) Chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and electrophysiological study of 92 cases. Brain 110: 1617–1630PubMedCrossRefGoogle Scholar
  83. 83.
    Gorson KC, Allam G, Ropper AH (1997) Chronic inflammatory demyelinating polyneuropathy: Clinical feature and response to treatment in 67 consecutive patients with and without a monoclonal gammopathy. Neurology 48: 321–328PubMedCrossRefGoogle Scholar
  84. 84.
    Dyck PJ, Lais AC, Ohta M et al. (1975) Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 50: 621–637PubMedGoogle Scholar
  85. 85.
    Barohn RJ, Kisser JT, Warmolts JR, Mendell JR (1989) Chronic inflammatory demyelinating polyradiculoneuropathy. Clinical characteristics, course, and recommendations for diagnostic criteria. Arch Neurol 46: 878–884PubMedCrossRefGoogle Scholar
  86. 86.
    Hughes RAC (1990) Guillain-Barrè syndrome. Springer, Berlin Heilderberg New YorkCrossRefGoogle Scholar
  87. 87.
    Cornblath DR, Chaudry V, Griffin JW (1991) Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Ann Neurol 30: 104–106PubMedCrossRefGoogle Scholar
  88. 88.
    Dyck PJ, Prineas J, Pollard J (1993) Chronic inflammatory demyelinatingpolyradiculoneuropathy. In: Dyck PJ, Thomas PK, Griffin JW, Low A, Poduslo JF (eds) Peripheral neuropathy, 3rd edn. WB Saunders, Philadelphia, pp 1498–1517Google Scholar
  89. 89.
    Ad Hoc Subcommittee of the American Academy of Neurology (1991) AIDS Task Force: Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Neurology 41: 617–618CrossRefGoogle Scholar
  90. 90.
    Albers J, Kelly J (1989) Aquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve 12: 435–451PubMedCrossRefGoogle Scholar
  91. 91.
    Simmons Z, Albers JW, Bromberg MB, Feldman EL (1995) Long term follow-up of patients with chronic inflammatory demyelinating polyneuropathy without and with monoclonal gammopathy. Brain 118: 359–368PubMedCrossRefGoogle Scholar
  92. 92.
    Nevo Y, Pestronk A, Kornberg AJ, Connolly AM et al. (1996) Childwood chronic inflammatory demyelinating neuropathies: clinical course and long term follow-up. Neurology: 47: 98–102PubMedCrossRefGoogle Scholar
  93. 93.
    Austin JH (1958) Recurrent polyneuropathies and their corticosteroid treatment. Brain 81: 157–192Google Scholar
  94. 94.
    Dyck PJ, O’Brien PC, Oviatt KF, Dinapoli RP et al. (1982) Prednisone improves chronic inflammatory demyelinating polyradiculoneuropathy more than no treatment. Ann Neurol 11: 136–141PubMedCrossRefGoogle Scholar
  95. 95.
    de Vivo DC, Engel WK (1970) Remarcable recovery of steroid-positive recurrent polineuropathy. J Neurol Neurosurg Psychiatry 33: 62–69CrossRefGoogle Scholar
  96. 96.
    Levy RL, Newkirk R, Ochoa J (1979) Treating chronic relapsing Guillain-Barrè syndrome by plasma exchange (letter). Lancet 2 (8136): 259–260PubMedCrossRefGoogle Scholar
  97. 97.
    Server AC, Lefkowith J, Braine H, McKhann GM (1979) Treatment of chronic relapsing inflammatory polyradiculoneuropathy by plasma exchange. Ann Neurol 6: 258–261PubMedCrossRefGoogle Scholar
  98. 98.
    Toyka KV, Augspach R, Wietholter H et al. (1982) Plasma exchange in chronic inflammatory polyneuropathy: evidence suggestive of pathogenic humoralfactor. Muscle Nerve 5: 479–484PubMedCrossRefGoogle Scholar
  99. 99.
    Dyck PJ, Daube J, O’Brien P et al. (1986) Plasma exchange in chronic inflammatory demyelinating polyneuropathy. N Engl J Med 314: 461–465PubMedCrossRefGoogle Scholar
  100. 100.
    Hahn Af, Bolton CF, Pillay N, Chalk C et al. (1996) Plasma-exchange therapy in chronic inflammatory demyelinating polyneuropathy. A double-blind sham controlled cross-over study. Brain 119: 1055–1066PubMedCrossRefGoogle Scholar
  101. 101.
    Heininger K, Gibbels K, Besinger UA, Borberg H et al. (1990) Role of therapeutic plasmapheresis in chronic inflammatory demyelinating polyradiculoneuropathy. In: Rock G (ed) Apheresis. Wiley-Liss, New York, pp 275–281Google Scholar
  102. 102.
    Thornton CA, Griggs RC (1994) Plasma exchange and intravenous immunoglobulin treatment of neuromuscular disease. Ann Neurol 35: 260–268PubMedCrossRefGoogle Scholar
  103. 103.
    Fanaroff AA, Korones SB, Wright LL et al. (1994) A controlled trial of intravenous immune globulin to reduce nosocomial infections in very-low-birth-weight infants. National Institute of Child Health and Human Development Neonatal Research Network. N Engl J Med 330: 1007–1013CrossRefGoogle Scholar
  104. 104.
    Schwarzt SA (1993) Clinical use of immune serum globulin as replacement therapy in patients with primary immunodeficiency syndromes. Clin Rev Allergy 10: 1–12Google Scholar
  105. 105.
    Ronda N, Hurez V, Kazatchine MD (1993) Intravenous immunoglobulin therapy of autoimmune and systemic inflammatory diseases. Vox Sang 65: 65–72CrossRefGoogle Scholar
  106. 106.
    Sullivan KM, Kopecky KJ, Jocom J et al. (1990) Immunomodulatory and antimicrobial efficacy of intravenous immunoglobulin in bone marrow transplantation. N Engl J Med 323: 705–712PubMedCrossRefGoogle Scholar
  107. 107.
    Hoang-Xuan K, Leger JM, Ben Younes-Chennoufi A et al. (1993) Traitement des neuropathies dysimmunitaires par immunoglobulines polyvalentes intraveineuses. Etude ouverte de 16 cases. Rev Neurol 149: 385–392PubMedGoogle Scholar
  108. 108.
    Faed JM, Day B, Pollock M et al. (1989) High-dose intravenous human immunoglobulin in chronic inflammatory demyelinating polyneuropathy. Neurology 39: 422–425PubMedCrossRefGoogle Scholar
  109. 109.
    Hodkinson SJ, Pollard JD, McLeod JG (1990) Cyclosporin A in the treatment of chronic demyelinating polyradiculoneuropathy. J Neurol Neurosurg Psychiatry 53: 327–330CrossRefGoogle Scholar
  110. 110.
    Nemni R, Amadio S, Fazio R et al. (1994) Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating neuropathy not responsive to other treatments. J Neurol Neurosurg Psychiatry 57(Suppl): 43–45PubMedCrossRefGoogle Scholar
  111. 111.
    Vermeulen M, van Doorn PA, Brand A et al. (1993) Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled study. J Neurol Neurosurg Psychiatry 56: 36–39PubMedCrossRefGoogle Scholar
  112. 112.
    Dyck PJ, Litchy WJ, Kratz KM et al. (1994) A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 36: 838–845PubMedCrossRefGoogle Scholar
  113. 113.
    Hahn AF, Bolton CF, Pillay N ET AL. (1996) Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy. A double-blind, placebo-controlled cross-over study. Brain 119: 1067–1077PubMedCrossRefGoogle Scholar
  114. 114.
    Kahn SN, Riches PG, Kohn J (1980) Paraproteinemia in neurological disease: incidence, associations, and classification of monoclonal immunoglobulins. J Clin Pathol 33: 617–621PubMedCrossRefGoogle Scholar
  115. 115.
    Kelly JJ, Kyle RA, O’Brien PC, Dyck PJ (1981) Prevalence of monoclonal protein in peripheralneuropathy. Neurology 31: 1480–1483PubMedCrossRefGoogle Scholar
  116. 116.
    Krol V, Straaten MJ, Ackerstaff RG, De Maat CE (1985) Peripheral plyneuropathy and monoclonal gammopathy of undetermined significance. J Neurol Neurosurg Psychiatry 48(7): 706–708CrossRefGoogle Scholar
  117. 117.
    Osby E, Noring L, Hast R, Kjellin KG, Knutsson E, Siden A (1982) Benign monoclonal gammopathy and peripheral neuropathy. Br J Haematol 51(4): 531–539Google Scholar
  118. 118.
    Vrethem M, Cruz M, Huang W et al. (1993) Clinical, neurophysiological and immunological evidence of polyneuropathy in patients with monoclonal gammopathies. J Neurol Sci 114: 193–199PubMedCrossRefGoogle Scholar
  119. 119.
    Nobile-Orazio E, Manfredini E, Carpo M et al. (1994) Frequency and clinical correlates of anti-neural IgM antibodies in neuropathy associated with IgM monoclonal gammopathy. Ann Neurol 36: 416–424PubMedCrossRefGoogle Scholar
  120. 120.
    Dalakas MC, Engel WK (1981) Polyneuropathy with monoclonal gammopathy: studies of 11 patients. Ann Neurol 10: 45–52PubMedCrossRefGoogle Scholar
  121. 121.
    Kelly JJ, Adelman E, Berkman E, Bhan I (1988) Polyneuropathies associated with IgM monoclonal gammopathies. Arch Neurol 45: 1355–1359PubMedCrossRefGoogle Scholar
  122. 122.
    Yeung KB, Thomas PK, King RHM et al. (1991) The clinical spectrum of peripheral neuropathies associated with benign monoclonal IgM, IgG and IgA paraproteinaemia. Comparative clinical, immunological and nerve biopsy findings. J Neurol 238: 383–391PubMedCrossRefGoogle Scholar
  123. 123.
    Nobile-Orazio E, Baldini L, Barbieri S et al. (1988) Treatment of patients with neuropathy and anti-MAG IgM M-proteins. Ann Neurol 24: 93–97PubMedCrossRefGoogle Scholar
  124. 124.
    Waterston JA, Brown MM, Ingram DA, Swash M (1992) Cyclosporin A therapy in paraprotein-associated neuropathy. Muscle Nerve 15: 445–448PubMedCrossRefGoogle Scholar
  125. 125.
    Dellagi K, Chedru F, Clauvel JP, Brouet JC (1984) Neuropathie periphérique de la macroglobulinemie de Waldenstrom. Presse Med 13: 1199–1201PubMedGoogle Scholar
  126. 126.
    Sherman WH, Olarte MR, McKiernan G et al. (1984) Plasma exchange treatment of peripheral neuropathy associated with plasma cell dyscrasia. J Neurol Neurosurg Psychiatry 47: 813–819PubMedCrossRefGoogle Scholar
  127. 127.
    Simmons Z, Bromberg MB, Feldman EL, Blaivas M (1993) Polyneuropathy associated with IgA monoclonal gammopathy of undetermined significance. Muscle Nerve 16: 77–83PubMedCrossRefGoogle Scholar
  128. 128.
    Dyck PJ, Low PA, Windebank AJ et al. (1991) Plasma exchange in polyneuropathy associated with monoclonal gammopathy of undetermined significance. N Engl J Med 325: 1482–1486PubMedCrossRefGoogle Scholar
  129. 129.
    Ellie E, Vital A, Steck A et al. (1996) Neuropathy associated with ‘benign’ anti-myelin-associated glycoprotein IgM gammopathy: clinical, immunological, neurophysiological, pathological findings and response to treatment in 33 cases. J Neurol 243: 34–43PubMedCrossRefGoogle Scholar
  130. 130.
    Oksenehendler E, Chevret S, Léger JM et al. (1995) Plasma exchange and chlorambucil in polyneuropathy associated with monoclonal IgM gammopathy. J Neurol Neurosurg Psychiatry 59: 243–247CrossRefGoogle Scholar
  131. 131.
    Cook D, Dalakas M, Galdi A et al. (1990) High-dose intravenous immunoglobulin in the treatment of demyelinating neuropathy associated with monoclonal gammopathy. Neurology 40: 212–214PubMedCrossRefGoogle Scholar
  132. 132.
    Léger JM, Younes-Chennoufi AB, Chassande B et al. (1994) Human immunoglobulin treatment of multifocal motor neuropathy and polyneuropathy associated with monoclonal gammopathy. J Neurol Neurosurg Psychiatry 57(suppl): 46–49PubMedCrossRefGoogle Scholar
  133. 133.
    Notermans NC, Lokhorst HM, Franssen H et al. (1996) Intermittent cyclophosphamide and prednisone treatment of polyneuropathy associated with monoclonal gammopathy of undetermined significance. Neurology 47(5): 1227–1233PubMedCrossRefGoogle Scholar
  134. 134.
    Blume G, Pestronk A, Goodnough LT (1995) Anti-MAG antibody-associated polyneuropathies: improvement following immunotherapy with monthly plasma-exchange and IV cyclophosphamide. Neurology 45(8): 1577–1580PubMedCrossRefGoogle Scholar
  135. 135.
    Kaji R, Hirota N, Oka N et al. (1994) Anti-GM1 antibodies and impaired blood-nerve barrier may interfere with remyelination in multifocal motor neuropathy. Muscle Nerve 17: 108–110PubMedCrossRefGoogle Scholar
  136. 136.
    Corse AM, Chaudry V, Crawford TO et al. (1996) Sensory nerve pathology in multifocal motor neuropathy. Ann Neurol 39: 319–325PubMedCrossRefGoogle Scholar
  137. 137.
    Lange DJ, Trojaborg W, Latov N et al. (1992) Multifocal motor neuropathy with conduction block: is it a distinct clinical entity? Neurology 43(3 Pt 1): 497–505CrossRefGoogle Scholar
  138. 138.
    Kornberg AJ, Pestronk A (1994) The clinical and diagnostic role of anti-GM1 antibody testing. Muscle Nerve 17(1): 100–104PubMedCrossRefGoogle Scholar
  139. 139.
    Feldman EL, Bromberg MB, Alberts JW, Pestronk A (1991) Immunosuppressive treatment in multifocal motor neuropathy. Ann Neurol 30: 397–401PubMedCrossRefGoogle Scholar
  140. 140.
    Krarup C, Stewart JD, Sumner AJ et al. (1990) A syndrome of asymmetric limb weakness with motor conduction block. Neurology 40: 118–127PubMedCrossRefGoogle Scholar
  141. 141.
    Parry GJ, Clarke S (1988) Multifocal acquired demyelinating neuropathy masquerading as motor neurondisease. Muscle Nerve 11: 103–107PubMedCrossRefGoogle Scholar
  142. 142.
    Pestronk A, Cornblath DR, Ilyas AA et al. (1988) A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol 24: 73–78PubMedCrossRefGoogle Scholar
  143. 143.
    Donaghy M, Mills KR, Boniface SJ et al. (1994) Pure motor demyelinating neuropathy: deterioration after steroid treatment and improvement with intravenous immunoglobulin. J Neurol Neurosurg Psychiatry 57(7): 778–783PubMedCrossRefGoogle Scholar
  144. 144.
    van den Berg LH, Lokhorst H, Wokke JH (1997) Pulsed high-dose dexamethasone is not effective in patients with multifocal neuropathy. Neurology 48(4): 1135PubMedCrossRefGoogle Scholar
  145. 145.
    Bouche P, Moulonguet A, Younes-Chennoufi AB et al. (1995) Multifocal motor neuropathy with conduction block: a study of 24 patients. J Neurol Neurosurg Psychiatry 59: 38–44PubMedCrossRefGoogle Scholar
  146. 146.
    Chaudry V, Corse AM, Cornblath DR et al. (1993) Multifocal motor neuropathy: response to human immune globulin. Ann Neurol 33: 237–242CrossRefGoogle Scholar
  147. 147.
    Comi G, Amadio S, Galardi G et al. (1994) Clinical and neurophysiological assessment of immunoglobulin therapy in five patients with multifocal motor neuropathy. J Neurol Neurosurg Psychiatry 57(suppl): 35–37PubMedCrossRefGoogle Scholar
  148. 148.
    Kaji R, Shibasaki H, Kimura J (1992) Multifocal demyelinating motor neuropathy: cranial nerve involvement and immunoglobulin therapy. Neurology 42: 506–509PubMedCrossRefGoogle Scholar
  149. 149.
    Nobile-Orazio E, Meucci N, Barbieri S et al. (1993) High-dose intravenousim-munoglobulin therapy in multifocal motor neuropathy. Neurology 43: 537–544PubMedCrossRefGoogle Scholar
  150. 150.
    Parry GJ (1996) AAEM case report # 30: multifocal motor neuropathy. Muscle Nerve 19: 269–276PubMedCrossRefGoogle Scholar
  151. 151.
    Azulay JP, Blin O, Pouget J et al. (1994) Intravenous immunoglobulin treatment in patients with motor neuron syndromes associated with anti-GM1 antibodies: a double-blind, placebo-controlled study. Neurology 44: 429–432PubMedCrossRefGoogle Scholar
  152. 152.
    van den Berg LH, Kerkoff H, Oey PL et al. (1995) Treatment of multifocal motor neuropathy with high dose intravenous immunoglobulins: a double-blind, placebo-controlled study. J Neurol Neurosurg Psychiatry 59: 248–252PubMedCrossRefGoogle Scholar
  153. 153.
    Azulay JP, Rihet P, Pouget J et al. (1997) Long term follow-up of multifocal motor neuropathy with conduction block under treatment. J Neurol Neurosurg Psychiatry 62(4): 391–394PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 1999

Authors and Affiliations

  • G. Comi
    • 1
  • L. Roveri
    • 1
  1. 1.Department of Clinical Neurophysiology, San Raffaele Scientific InstituteUniversity of MilanMilanItaly

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