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Sensory neuropathy with monoclonal IgM binding to a trisulfated heparin disaccharide
Author(s) -
Pestronk Alan,
Choksi Rati,
Logigian Eric,
AlLozi Muhammad T.
Publication year - 2003
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.10301
Subject(s) - polyneuropathy , disaccharide , immunoglobulin m , pathogenesis , chemistry , pathology , immunology , antibody , medicine , immunoglobulin g , biochemistry
We studied clinical and serological features of five patients with polyneuropathy and serum immunoglobulin M (IgM) binding to the trisulfated disaccharide IdoA2S‐GlcNS‐6S (TS‐HDS), the most abundant disaccharide component of heparin oligosaccharides. The patients all had painful, predominantly sensory polyneuropathies. Sensory loss was distal and panmodal. Electrophysiological and pathological studies were consistent with axonal loss, especially of unmyelinated axons. Immunohistochemistry showed IgM and κ light chains deposited around the rim of intermediate‐sized veins in the perimysium and epineurium. Serum IgM binding to TS‐HDS was selective, present in high titer (>12,000), and limited to κ light chains. We conclude that TS‐HDS is a newly identified target carbohydrate antigen of some IgM M‐proteins. Monoclonal IgM binding to TS‐HDS is associated with a painful, predominantly sensory, polyneuropathy syndrome with axonal loss and deposition of IgM in veins. The role of IgM binding to TS‐HDS in the pathogenesis of the neuropathy remains to be determined. Muscle Nerve 27: 188–195, 2003