Anti-myelin-associated glycoprotein antibodies and endoneurial cryoglobulin deposits responsible for a severe neuropathy.
A 73-year-old man was investigated for a peripheral neuropathy which occurred in the course of a Waldenstrom's macroglobulinemia. Serum immuno-fixation electrophoresis demonstrated two IgM monoclonal gammopathies of the kappa and lambda chain isotypes, and one had the physical characteristics of cryoglobulin. Immunoblot studies on the patient's serum revealed antibodies which reacted with peripheral nervous system proteins of different molecular weights including the myelin-associated glycoprotein (MAG). An immunofluorescence study of a superficial peroneal nerve biopsy revealed not only a binding of IgM and kappa light chain on several myelin sheaths but also the presence of IgM and kappa light chain deposits in the endoneurium. On electron microscopic examination, numerous fibres presented a widely spaced myelin and the endoneurial deposits had the ultrastructure of cryoglobulin. This is the first case presenting features of widely spaced myelin related to serum anti-MAG activity associated with monoclonal cryoglobulin deposits in the endoneurium.
Vital
A
A
Department of Neuropathology, Victor Segalen University, Bordeaux, France. anne.vital@neuropath.u-bordeaux2.fr
Favereaux
A
A
Martin-Dupont
P
P
Taupin
J L
JL
Petry
K
K
Lagueny
A
A
Canron
M H
MH
Vital
C
C
D000368:Aged; D001323:Autoantibodies / Q000097:blood*; D003450:Cryoglobulins / Q000032:analysis*; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009186:Myelin Sheath / Q000473:pathology / Q000648:ultrastructure; D018992:Myelin-Associated Glycoprotein / Q000276:immunology*; D010525:Peripheral Nerves / Q000473:pathology / Q000648:ultrastructure; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000276:immunology
D001323:Autoantibodies; D003450:Cryoglobulins; D018992:Myelin-Associated Glycoprotein