Anti-myelin-associated glycoprotein antibodies and endoneurial cryoglobulin deposits responsible for a severe neuropathy

• Published in: Acta neuropathologica Vol. 102; no. 4; pp. 409 - 412
• Main Authors: VITAL, Anne, FAVEREAUX, Alexandre, MARTIN-DUPONT, Philippe, TAUPIN, Jean-Luc, PETRY, Klaus, LAGUENY, Alain, CANRON, Marie-Hélène, VITAL, Claude
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01-10-2001; Springer Nature B.V
• Subjects: Aged; Antibodies; Autoantibodies - blood; Biological and medical sciences; Biopsy; Cryoglobulins - analysis; Glycoproteins; Humans; Immunodeficiencies. Immunoglobulinopathies; Immunofluorescence; Immunoglobulin M; Immunoglobulinopathies; Immunopathology; Isotypes; Macroglobulinemia; Male; Medical sciences; Microscopy, Electron; Monoclonal gammopathy; Myelin; Myelin Sheath - pathology; Myelin Sheath - ultrastructure; Myelin-associated glycoprotein; Myelin-Associated Glycoprotein - immunology; Nervous system; Peripheral Nerves - pathology; Peripheral Nerves - ultrastructure; Peripheral Nervous System Diseases - etiology; Peripheral Nervous System Diseases - immunology; Peripheral Nervous System Diseases - pathology; Peripheral neuropathy; Peroneal nerve; Physical characteristics; Ultrastructure; Waldenstrom Macroglobulinemia - complications; Waldenstrom Macroglobulinemia - immunology; Human; Immunopathology; Cryoglobulin; Nervous system diseases; Myelin; Glycoprotein; Male; Malignant hemopathy; Monoclonal antibody; Peripheral neuropathy; Case study; Immunoglobulinopathy; Lymphoproliferative syndrome; Complication; Peripheral nerve disease; Waldenstrom macroglobulinemia; Elderly
• ISSN: 0001-6322; 1432-0533
• DOI: 10.1007/s004010100381

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.