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Expert Clinical Perspectives: Paraproteinemic neuropathies
Expert Clinical Perspectives: Paraproteinemic neuropathies
Abstract: 
The diagnostic evaluation of a peripheral neuropathy includes testing for the presence of monoclonal gammopathy, which can be found in about 10% of patients with peripheral neuropathy. Our role, as physicians, is to determine whether the neuropathy is directly related to the gammopathy or whether the co-occurrence of these two disorders is purely coincidental. The evaluating physician needs to be familiar with
the different types of neuropathies associated with monoclonal gammopathies, their clinical and electrodiagnostic characteristics, and their appropriate diagnostic evaluation and management. Testing for monoclonal protein disorders includes serum protein electrophoresis (SPEP) and immunofixation of blood, and in some cases of urine, as well as measurement of free light chains and quantitative immunoglobulins. Specific antibody testing is directed by paraprotein type and neuropathy phenotype. Patients with abnormal free light chains in association with sensory and autonomic neuropathy should be evaluated for AL amyloidosis. When a lambda monoclonal protein is identified together with a clinical phenotype of chronic inflammatory demyelinating neuropathy (CIDP), a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome should be considered. Patients with IgM paraprotein associated neuropathy should be assessed for distal acquired demyelinating sensorimotor (DADS) neuropathy, with or without anti myelin associated glycoprotein (MAG) antibody or CANOMAD syndrome. In many cases, a monoclonal gammopathy of uncertain significance (MGUS) is incidental and unrelated to the neuropathy. Collaboration with oncology is critical in evaluating patients with monoclonal proteins to assess for underlying plasma cell neoplasms or B cell lymphomas.

The objectives of this activity are to: 1) Be able to order the appropriate diagnostic tests for paraproteinemias in patients with peripheral neuropathies undetermined etiology; (2) Understand the role of anti-MAG antibodies and vascular endothelial growth factor in the pathogenesis of paraproteinemic neuropathies, and be able to incorporate this information in an appropriate diagnostic evaluation; (3) Recognize and properly evaluated patients suspected of having neuropathies associated with AL amyloidosis, POEMS syndrome, DADS neuropathy, CANOMAD, and cryoglobulinemia.


ACCREDITATION STATEMENT
The AANEM is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

DISCLOSURE INFORMATION
Chafic Karam has received honoraria for consulting from Alexion Pharmaceuticals, Alnylam Pharmaceuticals, Alpine Pharmaceuticals,
Argenx, AstraZeneca, Corino Therapeutics, CSL Behring, Genetech, Ionis Pharmaceuticals, Neuroderm Ltd., Novo Nordisk, Pfizer, Sanofi,
Takeda Pharmaceutical Company, UCB, and Zai Lab Ltd. He has received research funding from Argenx, AstraZeneca, Genzyme, and Ionis Pharmaceuticals. Rebecca Traub has received research funding from Alnylam Pharmaceuticals, Argenx, Ionis Pharmaceuticals, and Pharnext and has received honoraria for consulting from Takeda Pharmaceuticals and UCB. Adam Cohen has received honoraria for consulting from Janssen, Genentech/Roche, Bristol Meyers Squibb, GlaxoSmithKline, Arcellx, Legend, Abbvie, Pfizer, Ichnos, and Iteos. He has received research funding from GlaxoSmithKline, Genentech and Novartis. Taha Qarni has no relevant disclosures. Conflicts have been resolved according to ACCME guidelines. 

CREDIT DESIGNATION
The AANEM is accredited by the American Council for Continuing Medical Education (ACCME) to providing continuing education for physicians. AANEM designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.  Credit expires  6/01/2027.
Author
Joome Suh, MD; Anthony A. Amato, MD.
Summary
Availability: On-Demand
Expires on Jun 01, 2027
Cost: Member: $0.00
Non-Member: $15.00
Credit Offered:
1 CME Credit
1 CEU Credit


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