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Journal Review: Anti–myelin-associated glycoprotein neuropathy: Where do we stand?
Journal Review: Anti–myelin-associated glycoprotein neuropathy: Where do we stand?

Myelin-associated glycoprotein (MAG) is a transmembrane glycoprotein concentrated in periaxonal Schwann cell and oligodendroglial membranes of myelin sheaths that serves as an antigen for immunoglobulin M (IgM) monoclonal antibodies. Individuals who harbor anti-MAG antibodies classically develop a progressive autoimmune peripheral neuropathy characterized clinically by ataxia, distal sensory loss, and gait instability, and electrophysiologically by distally accentuated conduction velocity slowing. Although off-label immunotherapy is common, there are currently no proven effective disease-modifying therapeutics, and most patients experience slow accumulation of disability over years and decades. The typically slowly progressive nature of this neuropathy presents unique challenges when trying to find effective anti-MAG therapeutic agents. Drug development has also been hampered by the lack of validated outcome measures that can detect clinically meaningful changes in a reasonable amount of time as well as by the lack of disease activity biomarkers. In this invited review, we provide an update on the state of clinicometric outcome measures and disease activity biomarkers in anti-MAG neuropathy. We highlight the insensitivity of widely used existing clinicometric outcome measures such as the Inflammatory Neuropathy Cause and Treatment (INCAT) disability score as well as the INCAT sensory subscore in anti-MAG neuropathy, referencing the two previous negative randomized controlled clinical trials evaluating rituximab. We then discuss newly emerging candidate therapeutic agents, including tyrosine kinase inhibitors and enhanced B-cell–depleting agents, among others. We conclude with a practical approach to the evaluation and management of anti-MAG neuropathy patients

Objectives: The objectives of this activity are to enable the reader to: 
1) Recognize and properly interpret the electrodiagnostic findings supportive of anti-MAG neuropathy;
2) Choose the appropriate clinical outcome measures and biomarkers for patients with anti-MAG neuropathy;
3) Implement best practices for management of anti-MAG neuropathy in clinical practice. 

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

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 11/7/2026.

A.S. has served as a consultant for Argenx and CSL Behring, and has received research funding from the GBS-CIDP Foundation and Bristol Myers Squibb. B.E. received research funding from Biogen, Genentech, Alexion, Pharnext, and Viela Bio, and served as a consultant for Biogen, Genentech, and Argenx. J.A. has served as a consultant for Alexion, Alnylym, Argenx, Akcea, Annexon, CSL Behring, Johnson & Johnson, Grifols, Takeda, Immunovant, Immunopharma, and Pfizer. Conflicts of interest have been resolved according to ACCME guidelines.

Amro Maher Stino MD; Bakri Elsheikh MBBS; Jeffrey A. Allen MD
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Expires on Nov 07, 2026
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