Neuropathies associated with monoclonal gammopathies

Alexandru C Barboi MD FACP (Dr. Barboi of NorthShore Medical Group has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (

Dr. Weimer of Columbia University has received consulting fees from Roche.

)
Originally released December 18, 2000; last updated December 2, 2017; expires December 2, 2020

This article includes discussion of neuropathies associated with monoclonal gammopathies, IgM associated polyneuropathy, IgG and IgA associated polyneuropathy, Waldenstrom macroglobulinemia polyneuropathy, multiple myeloma polyneuropathy, progressive amyloidosis polyneuropathy, and MAG antibody associated polyneuropathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

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 investigation of patients with polyneuropathy and monoclonal gammopathy is presented, based on 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, primary systemic sclerosis, and POEMS syndrome.

Key points

 

• Monoclonal gammopathy (IgG, IgM, or IgA) is identified in 10% of patients with idiopathic neuropathy. 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.

 

• 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 undetermined significance (MGUS)-associated neuropathy syndromes with autoantibody activity against peripheral nerve glycoproteins, such as myelin-associated glycoprotein (MAG), are generally IgM forms.

 

• Monoclonal gammopathy-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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