Evidence-based guideline: Intravenous
immunoglobulin in the treatment of
Report of the Therapeutics and Technology Assessment Subcommittee of the
American Academy of Neurology
H.S. Patwa, MD
V. Chaudhry, MD
H. Katzberg, MD
A.D. Rae-Grant, MD
Y.T. So, MD, PhD
Objective: To assess the evidence for the efficacy of IV immunoglobulin (IVIg) to treat neuromus-
Methods: The MEDLINE, Web of Science, and EMBASE databases were searched (1966–2009).
Selected articles were rated according to the American Academy of Neurology’s therapeutic clas-
sification of evidence scheme; recommendations were based on the evidence level.
Results and Recommendations: IVIg is as efficacious as plasmapheresis and should be offered for
treating Guillain-Barre ´ syndrome (GBS) in adults (Level A). IVIg is effective and should be offered
probably effective and should be considered for treating moderate to severe myasthenia gravis
and multifocal motor neuropathy (Level B). IVIg is possibly effective and may be considered for
treating nonresponsive dermatomyositis in adults and Lambert-Eaton myasthenic syndrome
(Level C). Evidence is insufficient to support or refute use of IVIg in the treatment of immunoglob-
ulin M paraprotein–associated neuropathy, inclusion body myositis, polymyositis, diabetic radicu-
loplexoneuropathy, or Miller Fisher syndrome, or in the routine treatment of postpolio syndrome
or in children with GBS (Level U). IVIg combined with plasmapheresis should not be considered for
treating GBS (Level B). More data are needed regarding IVIg efficacy as compared with other
treatments/treatment combinations. Most studies concluded IVIg-related serious adverse ef-
fects were rare. Given the variable nature of these diseases, individualized treatments depending
on patient need and physician judgment are important. Neurology®2012;78:1009–1015
AAN ? American Academy of Neurology; AE ? adverse effect; CI ? confidence interval; CIDP ? chronic inflammatory
demyelinating polyneuropathy; FDA ? Food and Drug Administration; GBS ? Guillain-Barre ´ syndrome; IBM ? inclusion body
myositis; IgM ? immunoglobulin M; INCAT ? Inflammatory Neuropathy Cause and Treatment Score; IVIg ? IV immunoglobulin;
LEMS ? Lambert-Eaton myasthenic syndrome; MG ? myasthenia gravis; MMN ? multifocal motor neuropathy; MP ? methyl-
prednisolone; MRC ? Medical Research Council; NDS ? neurologic disability scale; QMG ? Quantitative Myasthenia Gravis
score; RCT ? randomized controlled trial.
IV immunoglobulin (IVIg) is used to treat a range of
immune-mediated neurologic diseases. The US Food
and Drug Administration (FDA) approved IVIg for
use in Guillain-Barre ´ syndrome (GBS) and chronic
inflammatory demyelinating polyneuropathy (CIDP),
but IVIg use for non–FDA-approved indications is
common. Although IVIg appears to be well tolerated
in many patients, hypercoagulability and renal fail-
ure are of concern.
This American Academy of Neurology (AAN)
evidence-based guideline summarizes the evidence
and makes recommendations regarding IVIg use in
treating patients with neuromuscular disorders.
DESCRIPTION OF THE ANALYTIC PROCESS
The Therapeutics and Technology Assessment sub-
committee (appendix e-1 on the Neurology®Web
site at www.neurology.org) selected panelists on the
basis of expertise in IVIg use or familiarity with the
guideline process, or both. A literature search of
MEDLINE, Web of Science, and EMBASE data-
bases from 1966 to 2009 was conducted, using the
Correspondence & reprint
requests to American Academy
From the Yale University/VA Connecticut Healthcare System (H.S.P.), New Haven, CT; Johns Hopkins Medical Institute (V.C.), Baltimore, MD;
University of Toronto (H.K.), Toronto, Canada; Cleveland Clinic (A.D.R.-G.), Cleveland, OH; and Stanford University (Y.T.S.), Palo Alto, CA.
Approved by the Therapeutics and Technology Assessment Subcommittee on January 3, 2011; by the Practice Committee on February 7, 2011; and
by the AAN Board of Directors on December 12, 2011.
Disclosure: Author disclosures are provided at the end of the article.
Copyright © 2012 by AAN Enterprises, Inc.
H.S. Patwa, V. Chaudhry, H. Katzberg, et al.
Subcommittee of the American Academy of Neurology
neuromuscular disorders: Report of the Therapeutics and Technology Assessment
Evidence-based guideline: Intravenous immunoglobulin in the treatment of
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