Immune treatment for a peripheral neuropathy due to an IgM paraprotein (an abnormal amount of antibody or immunoglobulin in the blood) which may be an antibody against a protein on the myelin sheath of nerve cells (myelin-associated glycoprotein)

Lunn MPT, Nobile-Orazio E

Published Online:

October 8, 2008

An IgM paraprotein in the blood may be an antibody against myelin-associated glycoprotein and may damage nerve fibres and cause a peripheral neuropathy. Treatments that act on the immune system such as plasma exchange (which removes circulating antibodies and replaces blood plasma with a clean plasma substitute), intravenous immunoglobulin (antibodies that have been purified from donated blood), corticosteroids or chemotherapeutic therapies might be expected to reduce levels of these antibodies and slow or prevent progression of the disease. Many of these therapies have been tried but we found only five small randomised controlled trials, including 97 participants, of adequate quality. Two trials with 33 participants (20 with antibodies against myelin-associated glycoprotein) suggest that intravenous immunoglobulin may sometimes produce short-term benefit and is relatively safe. Severe adverse effects were not reported in these trials. The other trials do not allow conclusions about the efficacy of other agents. Large, well-designed randomised trials are needed to assess the efficacy of the existing or novel therapies.

Abstract (click to read)

Background:

Serum monoclonal anti-myelin associated glycoprotein antibodies may be pathogenic in some people with IgM paraprotein and demyelinating neuropathy. Immunotherapies aimed at reducing the level of these antibodies might be expected to be beneficial.

Objectives:

To examine the efficacy of any form of immunotherapy in reducing disability and impairment resulting from IgM anti-myelin associated glycoprotein paraprotein-associated demyelinating peripheral neuropathy.

Search strategy:

We searched the Cochrane Neuromuscular Disease Group Register (March 2005), MEDLINE (January 1966 to March 2005) and EMBASE (January 1980 to March 2005) for controlled trials. We also checked bibliographies and contacted authors and experts in the field.

Selection criteria:

We included randomised or quasi-randomised controlled trials of participants of any age treated with any type of immunotherapy for anti-myelin-associated glycoprotein antibody associated demyelinating peripheral neuropathy with monoclonal gammopathy of undetermined significance of any severity. Our primary outcome measure was change in the Neuropathy Impairment Scale or Modified Rankin Scale at six months after randomisation
Secondary outcome measures were: Neuropathy Impairment Scale or the Modified Rankin Score at 12 months after randomisation; ten-metre walk time, subjective clinical scores and electrophysiological parameters at six and 12 months after randomisation; IgM paraprotein levels and anti-myelin associated glycoprotein antibody titres at six months after randomisation and adverse effects of treatments.

Data collection and analysis:

We identified eight possible trials. Of these, five randomised controlled trials were included after discussion between the authors. One author extracted and the other checked the data. No missing data could be obtained from trial authors.

Main results:

The five eligible trials (97 participants) tested intravenous immunoglobulin, interferon-alpha or plasma exchange. Only two, of intravenous immunoglobulin, had comparable interventions and outcomes but both were short-term.

There were no significant benefits of the treatments used in the outcomes predefined for this review, but not all the predefined outcomes were used in every included trial. Intravenous immunoglobulin showed benefits in terms of improvement in Modified Rankin Scale at two weeks and 10-metre walk time at four weeks. Serious adverse effects of intravenous immunoglobulin are known to occur from observational studies but none were encountered in these trials.

Authors' conclusions:

There is inadequate reliable evidence from trials of immunotherapies in anti-myelin associated glycoprotein paraproteinaemic neuropathy to recommend any particular immunotherapy treatment. Intravenous immunoglobulin is relatively safe and may produce some short-term benefit. Large well-designed randomised trials of at least six to 12 months duration are required to assess existing or novel therapies.

This record should be cited as:

Lunn MPT, Nobile-Orazio E. Immunotherapy for IgM anti-myelin-associated glycoprotein paraprotein-associated peripheral neuropathies. Cochrane Database of Systematic Reviews 2006, Issue 2. Art. No.: CD002827. DOI: 10.1002/14651858.CD002827.pub2

Assessed as up to date:

January 2, 2006

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Primary Review Group:

Neuromuscular Disease Group

Health topics:

Neurology > Peripheral neuropathy > Paraproteinaemic neuropathy

Cochrane Summaries^beta

Independent high-quality evidence for health care decision making

Immune treatment for a peripheral neuropathy due to an IgM paraprotein (an abnormal amount of antibody or immunoglobulin in the blood) which may be an antibody against a protein on the myelin sheath of nerve cells (myelin-associated glycoprotein)

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