Focal myelin swellings and tomacula in anti-MAG IgM paraproteinaemic neuropathy: Novel teased nerve fiber studies

Focal myelin swellings and tomacula in teased nerve fibers from a case of IgM anti-myelin-associated glycoprotein (MAG) paraproteinaemic neuropathy were examined using a novel technique. Five different morphologic abnormalities were identified-myelin sheath outfolding, myelin sheath infolding, enlargement of the adaxonal space, myelin degeneration, multiple increased concentric loops-and a combination of these structural abnormalities often occur in association with myelin degeneration. Similar structural changes were found in externally normal segments of teased fibers without evidence of myelin swelling or tomacula from the same case. These structural abnormalities are consistent with a disturbance of the normal adhesion functions of MAG in the maintenance of axon-myelin relationships.