IVIG Treatment and Prognosis in Guillain–Barré Syndrome
Guillain–Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). However, about 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months. Important clinical factors associated with poor outcome are age, presence of preceding diarrhea and the severity of disability in the early course of disease. These clinical factors were combined in a clinical prognostic scoring scale, the Erasmus GBS Outcome Scale (EGOS).
Materials and Methods
GBS patients being unable to walk unaided are currently treated with a standard single IVIg dose (0.4 g/kg bodyweight for 5 days). A recent retrospective study in 174 GBS patients enrolled in one of our randomized controlled clinical trials showed that patients with a minor increase of serum IgG level after standard single IVIg dose recovered significantly slower. Additionally, fewer patients reached the ability to walk unaided at six months after correction for the known clinical prognostic factors (multivariate analysis; P < 0.022).
It is yet unknown why some GBS patients only have a minor increase after standard IVIg treatment. By using the EGOS it is possible to select GBS patients with a poor prognosis. These patients potentially may benefit from a second IVIg dose.
A standard dose of IVIG is not sufficiently effective in many GBS patients. Whether these patients might benefit from a second IVIg dose needs further investigation.
- Hughes, RA, Cornblath, DR (2005) Guillain–Barré syndrome. Lancet 366: pp. 1653-1666 CrossRef
- Doorn, PA, Ruts, L, Jacobs, BC (2008) Clinical features, pathogenesis, and treatment of Guillain–Barré syndrome. Lancet Neurol 7: pp. 939-950 CrossRef
- Jacobs, BC, Rothbarth, PH, Meché, FG, Herbrink, P, Schmitz, PI, Klerk, MA (1998) The spectrum of antecedent infections in Guillain–Barré syndrome: a case-control study. Neurology 51: pp. 1110-1115
- Asbury, AK, Cornblath, DR (1990) Assessment of current diagnostic criteria for Guillain–Barré syndrome. Ann Neurol 27: pp. S21-S24 CrossRef
- Willison, HJ (2005) The immunobiology of Guillain–Barré syndromes. J Peripher Nerv Syst 10: pp. 94-112 CrossRef
- Halstead, SK, O'Hanlon, GM, Humphreys, PD, Morrison, DB, Morgan, BP, Todd, AJ (2004) Anti-disialoside antibodies kill perisynaptic Schwann cells and damage motor nerve terminals via membrane attack complex in a murine model of neuropathy. Brain 127: pp. 2109-2123 CrossRef
- Susuki, K, Rasband, MN, Tohyama, K, Koibuchi, K, Okamoto, S, Funakoshi, K (2007) Anti-GM1 antibodies cause complement-mediated disruption of sodium channel clusters in peripheral motor nerve fibers. J Neurosci 27: pp. 3956-3967 CrossRef
- Halstead, SK, Zitman, FM, Humphreys, PD, Greenshields, K, Verschuuren, JJ, Jacobs, BC (2008) Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 131: pp. 1197-1208 CrossRef
- Hughes RA, Raphael JC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain–Barré syndrome. Cochrane Database Syst Rev. CD002063, 2006.
- Raphael JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain–Barré syndrome. Cochrane Database Syst Rev. CD001798, 2002.
- Meché, FG, Schmitz, PI (1992) A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain–Barré syndrome. Dutch Guillain–Barré Study Group. N Engl J Med 326: pp. 1123-1129 CrossRef
- Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain–Barré syndrome. Lancet 349: pp. 225-230 CrossRef
- Hughes, RA, Swan, AV, Raphael, JC, Annane, D, Koningsveld, R, Doorn, PA (2007) Immunotherapy for Guillain–Barré syndrome: a systematic review. Brain 130: pp. 2245-2257 CrossRef
- Koningsveld, R, Schmitz, PI, Meché, FG, Visser, LH, Meulstee, J, Doorn, PA (2004) Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain–Barré syndrome: randomised trial. Lancet 363: pp. 192-196 CrossRef
- Koningsveld, R, Schmitz, PI, Ang, CW, Groen, J, Osterhaus, AD, Meché, FG (2002) Infections and course of disease in mild forms of Guillain–Barré syndrome. Neurology 58: pp. 610-614
- Durand, MC, Porcher, R, Orlikowski, D, Aboab, J, Devaux, C, Clair, B (2006) Clinical and electrophysiological predictors of respiratory failure in Guillain–Barré syndrome: a prospective study. Lancet Neurol 5: pp. 1021-1028 CrossRef
- Koningsveld, R, Steyerberg, EW, Hughes, RA, Swan, AV, Doorn, PA, Jacobs, BC (2007) A clinical prognostic scoring system for Guillain–Barré syndrome. Lancet Neurol 6: pp. 589-594 CrossRef
- Farcas, P, Avnun, L, Frisher, S, Herishanu, YO, Wirguin, I (1997) Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain–Barré syndrome. Lancet 350: pp. 1747 CrossRef
- Kuitwaard, K, Gelder, J, Tio-Gillen, AP, Hop, WC, Gelder, T, Toorenenbergen, AW (2009) Pharmacokinetics of intravenous immunoglobulin and outcome in Guillain–Barré syndrome. Ann Neurol 66: pp. 597-603 CrossRef
- Cornblath, DR, Hughes, RA (2009) Treatment for Guillain–Barré syndrome. Ann Neurol 66: pp. 569-570 CrossRef
- IVIG Treatment and Prognosis in Guillain–Barré Syndrome
- Open Access
- Available under Open Access This content is freely available online to anyone, anywhere at any time.
Journal of Clinical Immunology
Volume 30, Issue 1 Supplement, pp 74-78
- Cover Date
- Print ISSN
- Online ISSN
- Springer US
- Additional Links
- Guillain–Barré syndrome
- Industry Sectors
- Author Affiliations
- 1. Department of Neurology, Erasmus MC, University Medical Center Rotterdam, room BA 450, ‘s-Gravendijkwal 230, 3015, CJ, Rotterdam, The Netherlands
- 2. Department of Neurology, Erasmus MC, University Medical Center Rotterdam, ‘s-Gravendijkwal 230, 3015, CJ, Rotterdam, The Netherlands
- 3. Elkerliek Ziekenhuis, Helmond, The Netherlands
- 4. Department of Immunology, Erasmus MC, University Medical Center Rotterdam, Rotterdam, The Netherlands