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Review
. 2009 Dec;158 Suppl 1(Suppl 1):34-42.
doi: 10.1111/j.1365-2249.2009.04025.x.

Clinical applications of intravenous immunoglobulins in neurology

R A C Hughes  1 , M C Dalakas, D R Cornblath, N Latov, M E Weksler, N Relkin
Affiliations
Review

Clinical applications of intravenous immunoglobulins in neurology

R A C Hughes et al. Clin Exp Immunol. 2009 Dec.

Abstract

Intravenous immunoglobulin (IVIg) is used increasingly in the management of patients with neurological conditions. The efficacy and safety of IVIg treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and Guillain-Barré syndrome (GBS) have been established clearly in randomized controlled trials and summarized in Cochrane systematic reviews. However, questions remain regarding the dose, timing and duration of IVIg treatment in both disorders. Reports about successful IVIg treatment in other neurological conditions exist, but its use remains investigational. IVIg has been shown to be efficacious as second-line therapy in patients with dermatomyositis and suggested to be of benefit in some patients with polymyositis. In patients with inclusion body myositis, IVIg was not shown to be effective. IVIg is also a treatment option in exacerbations of myasthenia gravis. Studies with IVIg in patients with Alzheimer's disease have reported increased plasma anti-Abeta antibody titres associated with decreased Abeta peptide levels in the cerebrospinal fluid following IVIg treatment. These changes at the molecular level were accompanied by improved cognitive function, and large-scale randomized trials are under way.

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Figures

Fig. 1
Fig. 1
Anosmin-1 (KAL-1) is down-regulated in dermatomyositis (DM) but not inclusion body myositis (IBM) after intravenous immunoglobulin. Microarray results.
Fig. 2
Fig. 2
High serum amyloid-beta (Aβ)-autoantibody levels are associated with decreased diffuse plaques in Alzheimer's disease (AD). AD patients were allocated to a group with high plaque labelling index [high immunoglobulin (Ig)G] and one with low plaque labelling index (low IgG). Patients with low plaque scores (low IgG) harboured more diffuse plaques, but not neuritic plaques than patients with high plaque scores (high IgG). Figure reproduced with permission from [66].
Fig. 3
Fig. 3
Cerebrospinal fluid (CSF) amyloid-beta (Aβ) decreases during intravenous immunoglobulin (IVIg) treatment. Both CSF Aβ40 and Aβ42 levels are decreased significantly following IVIg treatment for 6 months. When IVIg treatment was stopped (wash-out), Aβ peptide levels in the CSF receded to their pretreatment levels, but improved again when treatment was reinitiated after 3 months. *Significant difference from baseline by t-test at P< 0·005.
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