Peripheral Neuropathies Associated With Monoclonal Gammopathies

Elie Naddaf, MD; Michelle L. Mauermann, MD, FAAN Peripheral Nerve and Motor Neuron Disorders p. 1369-1383 October 2020, Vol.26, No.5 doi: 10.1212/CON.0000000000000919
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PURPOSE OF REVIEW Neurologists commonly evaluate patients with a monoclonal gammopathy and peripheral neuropathy. As both monoclonal gammopathy and peripheral neuropathy are common in the general population, their coexistence may, in some instances, be purely coincidental. However, monoclonal gammopathies or underlying lymphoplasmacytic disorders can affect the peripheral nervous system by various mechanisms. This article discusses how to approach patients with monoclonal gammopathy and peripheral neuropathy, highlighting clinical and laboratory clues that may aid in establishing a diagnosis in a timely manner.

RECENT FINDINGS From a hematologic standpoint, a monoclonal gammopathy may be of undetermined significance or can be associated with an underlying myeloma, lymphoplasmacytic lymphoma, or amyloidosis. Each of these conditions can cause peripheral neuropathy, with varying clinical and electrodiagnostic profiles. Treatment usually consists of treating the underlying hematologic disorder. IgM-associated peripheral neuropathy may not require treatment from a hematologic standpoint, and only anecdotal evidence exists for the use of immunotherapy in such patients. Therefore, treatment should be determined on a case-by-case basis.

SUMMARY Evaluating for an association between a monoclonal gammopathy and a peripheral neuropathy in a patient depends on the monoclonal gammopathy subtype and the clinical and electrodiagnostic characteristics of the peripheral neuropathy.

Address correspondence to Dr Elie Naddaf, Mayo Clinic, 200 First St SW, Rochester, MN 55905,

RELATIONSHIP DISCLOSURE: Dr Naddaf reports no disclosure. Dr Mauermann serves on the editorial board of Mayo Clinic Proceedings; receives research/grant support from Alnylam Pharmaceuticals, Inc, and Ionis Pharmaceuticals, Inc; and receives publishing royalties from Oxford University Press.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Drs Naddaf and Mauermann discuss the unlabeled/investigational use of IV immunoglobulin and rituximab for the treatment of IgM neuropathy.

© 2020 American Academy of Neurology.