IgM antibodies to N-acetylgalactosaminyl GD1a in benign monomelic amyotrophy of the lower limb

A male Caucasian patient is presented with a greater than 10-year history of painless weakness of the right leg, stable over the last 7 years. His examination demonstrated severe wasting and moderate weakness of his right calf muscle. Electrodiagnostic study revealed acute denervation of the right calf and chronic partial denervation of multiple right leg muscles. MRI demonstrated atrophy and fatty infiltration of the right calf muscles. Based on previous clinical, electrophysiologic, and radiologic descriptions of this disorder, a diagnosis was made of benign monomelic amyotrophy of the lower limb. Serologic examination for anti-glycoconjugate antibodies revealed high titers of IgM antibodies with selective binding to the disialoganglioside N-acetylgalactosaminyl GD1a (GalNAcGD1a). Testing for antibodies to GalNAcGD1a should be considered in patients with this phenotype. Their presence could suggest a role for immunomodulatory therapy.