Polyneuropathy and IgM monoclonal gammopathy: studies on the pathogenetic role of anti-myelin-associated glycoprotein antibody.
Attention has recently been directed toward patients having a polyneuropathy and a monoclonal IgM anti-myelin-associated glycoprotein (anti-MAG) antibody. The possibility of a pathogenetic role for the anti-MAG antibody in the evolution of the polyneuropathy and in the development of central nervous system signs, including tremor and ataxia, remains unresolved. In 5 patients with this syndrome whose clinical courses were followed closely, in 1 of whom a complete postmortem examination of the nervous system was performed, we made the following observations: the anti-MAG antibody did not localize to the compact layer of the myelin sheath in affected nerves, but did localize to areas of myelin splitting; anti-MAG antibody present in the sural nerve of an affected individual for 7 years was not associated with progressive pathology; anti-MAG antibody was not deposited in the central nervous system of an affected individual, although the antibody did bind to these same tissues in vitro; deposition of anti-MAG antibody observed at postmortem examination did not correlate with the degree of pathological change; and study of the peripheral nervous system favored a primary axonal neuropathy with secondary demyelination.
Mendell
J R
JR
Sahenk
Z
Z
Whitaker
J N
JN
Trapp
B D
BD
Yates
A J
AJ
Griggs
R C
RC
Quarles
R H
RH
D000328:Adult; D000368:Aged; D001323:Autoantibodies; D005455:Fluorescent Antibody Technique; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology* / Q000473:pathology; D007124:Immunoenzyme Techniques; D007075:Immunoglobulin M; D008297:Male; D008875:Middle Aged; D009185:Myelin Proteins / Q000276:immunology*; D018992:Myelin-Associated Glycoprotein; D009420:Nervous System / Q000473:pathology; D010525:Peripheral Nerves / Q000276:immunology / Q000473:pathology; D010523:Peripheral Nervous System Diseases / Q000276:immunology* / Q000473:pathology
D001323:Autoantibodies; D007075:Immunoglobulin M; D009185:Myelin Proteins; D018992:Myelin-Associated Glycoprotein