This article includes discussion of neuropathies associated with monoclonal gammopathies, IgM demyelinating neuropathy, nonmalignant IgG and IgA monoclonal gammopathy and neuropathy, nonmalignant IgM monoclonal gammopathy, Waldenstrom macroglobulinemia, and anti-MAG syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.
Monoclonal gammopathy is identified in 10% of patients with idiopathic peripheral neuropathy, and the presence of a monoclonal protein has implications regarding the etiology, progression, and prognosis of the neuropathy. In this article, the authors review the wide spectrum of malignant and nonmalignant monoclonal gammopathies, and the relevant associated systemic diseases. A rational approach to investigation of patients with polyneuropathy and monoclonal gammopathy is presented, based on the clinical and electrophysiological features, as well as the quantity and subtype of monoclonal protein. Finally, the most current therapies are reviewed, including recent treatment trials for anti-MAG neuropathy, multiple myeloma, and POEMS syndrome.
• Monoclonal gammopathy (IgG, IgM, or IgA) is identified in 10% of patients with idiopathic neuropathy.
• Peripheral neuropathy and monoclonal gammopathy may be the presenting features of a plasma cell dyscrasia.
• Patients with nonmalignant monoclonal gammopathy should be followed carefully; malignant transformation occurs at a rate of 1.5% per year.
• Monoclonal gammopathy of undetermined significance (MGUS)-associated neuropathy syndromes with autoantibody activity against peripheral nerve glycoproteins, such as myelin-associated glycoprotein (MAG), are generally IgM forms.
• MGUS-associated peripheral neuropathy is potentially treatable; however, the preferred treatment agent depends on the subtype of monoclonal protein and the presence or absence of an underlying plasma cell dyscrasia.
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