title: An update on monoclonal gammopathy and neuropathy.
abstract: Peripheral neuropathy associated with monoclonal gammopathy is a rare but important cause of neuropathy that can herald serious underlying disease. IgM monoclonal gammopathy of undetermined significance (MGUS) is the most commonly found monoclonal gammopathy associated with neuropathy, with characteristic clinical, electrophysiologic, and pathologic features. The IgG and IgA monoclonal gammopathies are rarely associated with specific neuropathies. Standard immunomodulatory agents including steroids, intravenous immunoglobulin, and plasmapheresis have shown limited efficacy in IgM MGUS. Neuropathies associated with specific lymphoproliferative disorders may not respond to treatments aimed at that disorder. Case series had shown promising results with rituximab, a monoclonal antibody that targets the B cell surface antigen CD20 and results in a rapid and sustained depletion of B cells; however, two recent randomized controlled trials with rituximab failed to provide evidence of efficacy in primary outcome measures, despite reduction in antibody levels. Long-term studies looking at the association between specific immunologic markers and disease recurrence are needed to ultimately develop targeted therapies.
authors.affiliation: Department of Neurology, Wayne State University-Detroit Medical Center, 4201 St. Antoine UHC 8C.28, Detroit, MI 48201, USA. sramchan@med.wayne.edu
authors.forename: Sindhu
authors.initials: S
authors.lastname: Ramchandren
authors.forename: Richard A
authors.initials: RA
authors.lastname: Lewis
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use; D010265:Paraproteinemias / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007155:Immunologic Factors; D000069283:Rituximab
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