AIDP and CIDP having specific antibodies to the carbohydrate epitope (-NeuAcalpha2-8NeuAcalpha2-3Galbeta1-4Glc-) of gangliosides.

Abstract

Anti-ganglioside antibodies were investigated in plasma exchange solutions (PEs) from two patients with acute and chronic inflammatory demyelinating neuropathies (AIDP and CIDP). Both cases show markedly elevated antibody titers against the lacto-series gangliosides, GM3, GD3, and GT3. In the CIDP patient, the IgG antibody titer to GD3 was remarkably elevated (titer, 1:10,000), indicating maximal avidity to the tetrasaccharide epitope (-NeuAcalpha2-8NeuAcalpha2-3Galbeta1-4Glc-). There were also activities toward GM4 and GM2 with the affinity higher to GM4 than to GM2, indicating that the antibody activity was not highly specific. In contrast, the antibody activities in the AIDP patient showed similar avidity to GM3, GD3, and GT3. These two patients are very rare cases that have not previously encountered in GBS. The effects on co-cultured cells of rat spinal cord and muscle differed according to which PE was used. PE from the AIDP patient produced an inhibitory effect (reduction to 26.8%) on the spontaneous muscle action potential of the neuromuscular junction (NMJ), but the PE from the CIDP patient did not. Thus, in AIDP, the common epitope of GM3, GD3, or GT3 may be shared with certain antigens localized in the peripheral nervous system (PNS) and may participate in a component of conduction-related molecules in the NMJ. High titers of anti-GD3 antibody and the distortion of antibody recognition found in CIDP seem to have no immediate effect on electrophysiologic function in the PNS.