ganglioside--gd1b and Syndrome

We describe the clinical course, with special attention to the disturbance of eye movements, of a 29-year-old man with chronic ataxic neuropathy with ophthalmoplegia, IgM paraprotein, cold agglutinins and anti-GD1b disialosyl antibodies (CANOMAD). Using the magnetic search coil technique, we documented convergence during upward saccades and other features suggestive of dorsal midbrain syndrome. Thus, in common with Miller Fisher syndrome, CANOMAD may present with clinical findings implicating involvement of the central nervous system, which contains ganglioside antigens to anti-GD1b antibodies.

Topics: Adult; Anemia, Hemolytic, Autoimmune; Antibodies, Monoclonal; Antibodies, Monoclonal, Murine-Derived; Autoantibodies; Diagnosis, Differential; Gait Ataxia; Gangliosides; Humans; Immunoglobulin M; Male; Mesencephalon; Neurologic Examination; Ophthalmoplegia; Paraproteinemias; Plasma Exchange; Rituximab; Syndrome

Topics: Antibodies, Anti-Idiotypic; Gangliosides; Humans; Influenza Vaccines; Influenza, Human; Male; Middle Aged; Motor Neuron Disease; Paralysis; Syndrome

We demonstrated that an IgM M-protein from a patient with motor neuron syndrome had antibody activity against gangliosides GM1, GD1b, and asialo GM1. Studies with a sugar-binding lectin suggested that the epitope in the patient's M-IgM involved the Gal(beta 1-3) GalNAc moiety. Immunohistological techniques demonstrated staining of axons in the lumbar roots, granular cells, and white matter in the cerebellum by the patient's M-IgM. We propose that, in this case, an autoimmune mechanism of motor neuron syndrome associated with a monoclonal protein is most likely.

Topics: Antibodies, Monoclonal; Autoantibodies; Brain; Female; G(M1) Ganglioside; Gangliosides; Humans; Middle Aged; Motor Neurons; Neuromuscular Diseases; Spinal Cord; Syndrome