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  • Updated 10.18.2023
  • Released 12.18.2000
  • Expires For CME 10.18.2026

Monoclonal gammopathy of neurologic significance



Monoclonal gammopathy may occur in 1% of normal people over the age of 50, 1.7% over the age of 70, and 6% over the age of 90 (111). Monoclonal gammopathy is identified in 10% of patients with idiopathic peripheral neuropathy, and the presence of a monoclonal protein may have significant implications regarding the etiology, progression, and prognosis of the neuropathy. In this article, the author reviews the wide spectrum of malignant and nonmalignant monoclonal gammopathies and the relevant associated systemic diseases. A rational approach to the 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 treatment trials for anti-MAG neuropathy, multiple myeloma, Waldenstrom macroglobulinemia, cryoglobulinemic neuropathy, and POEMS syndrome.

Key points

• Monoclonal gammopathy (IgG, IgM, or IgA) is identified in 10% of patients with idiopathic polyneuropathy. Approximately half of these associations are coincidental given the prevalence of monoclonal gammopathies in the general population.

• Peripheral neuropathy and monoclonal gammopathy may be the presenting features of a plasma cell dyscrasia particularly if the monoclonal protein is IgM: monoclonal gammopathy of clinical significance (MGCS).

• Patients with nonmalignant monoclonal gammopathy should be followed carefully; malignant transformation occurs at a rate of 1.5% per year. In all monoclonal gammopathies, but particularly in light chain types associated polyneuropathy, it is important to rule out primary systemic amyloidosis or POEMS early in the course.

• Monoclonal gammopathy of neurologic significance (MGNS) with autoantibody activity against peripheral nerve glycoproteins, such as myelin-associated glycoprotein (MAG), are generally IgM forms.

• Monoclonal gammopathy of neurologic significance (MGNS) 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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