Characterization of neuropathies associated with elevated IgM serum levels

Abstract

Background: In contrast to the IgM monoclonal gammopathies the neuropathy associated with polyclonal IgM gammopathy has not been well characterized.

Objective: To characterize the neuropathy in patients with elevated serum IgM.

Design: Retrospective review.

Setting: Academically based neuropathy center.

Patients: 45 patients with elevated quantitative immunoglobulin M were identified.

Main outcome measures: Patients are described with regard to clinical phenotype, electrodiagnostic features of demyelination or focality, presence of IgM monoclonal gammopathy, and presence of autoantibody activity.

Results: Elevated IgM levels occurred in 45 (11.5%) of 391 patients. Of these, 24 (53%) had polyclonal gammopathy and 21 (47%) had an IgM monoclonal gammopathy. Anti-nerve antibodies occurred in 14/21 (67%) of patients with monoclonal gammopathy, as compared to 1/24 (4%) with polyclonal gammopathy. Clinically, most patients in all groups had a predominantly large fiber sensory neuropathy. Thirty patients underwent electrodiagnostic testing. Of these, 22/30 (73%) fulfilled at least one published criteria for CIDP, including 92% of the monoclonal gammopathy patients and 59% of the polyclonal gammopathy patients. Fifteen of the 30 patients had evidence of focality or multifocality, with 14 of these 15 showing evidence of demyelination.

Conclusions: Monoclonal and polyclonal IgM patients have similar distributions of neuropathy phenotypes. Neuropathy in association with elevated serum IgM, with or without monoclonal gammopathy or autoantibody activity, is more likely to be demyelinating or multifocal. Serum quantitative IgM level and immunofixation in neuropathy patients may aid in identification of an immune mediated or a demyelinating component.