gq1b-ganglioside and Polyneuropathies

We performed a serological investigation using glycoarray in Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and multifocal motor neuropathy (MMN). Antibodies to 10 glycolipids and 45 glycolipid complexes were tested. Anti-GM1/sulfatide and anti-GA1/sulfatide IgG antibodies were common in GBS (20.0% and 19.0%, respectively). Anti-GQ1b/sulfatide IgG antibody was detected in 14.0% of GBS patients. IgG antibodies to antigens containing GQ1b were significantly correlated with ophthalmoplegia in GBS (p<0.01). IgM antibodies to antigens containing GM1 or GalNAc-GD1a were in 50% and 37.5% of MMN patients, respectively. Glycoarray is efficient for detecting antibodies against numerous glycolipid complexes in immune-mediated neuropathies.

Topics: Adult; Aged; Aged, 80 and over; Autoantibodies; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Japan; Male; Middle Aged; Polyneuropathies; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Statistics, Nonparametric; Sulfoglycosphingolipids; Young Adult

In the present report, an unusual case of recurrent Miller Fisher syndrome is described. The patient presented within ten years three similar episodes of ophthalmoplegia, ataxia and areflexia associated with oropharyngeal weakness and signs of mild distal sensory neuropathy. An elevated titer of anti-GQ1b ganglioside antibodies correlated well with the clinical symptoms and signs. The pathogenic role of these antibodies in Miller Fisher syndrome is discussed.

Topics: Autoantibodies; Cerebellar Ataxia; Follow-Up Studies; Gangliosides; Humans; Male; Middle Aged; Nerve Growth Factors; Neurologic Examination; Ophthalmoplegia; Polyneuropathies; Recurrence; Reflex, Abnormal

A 43-year-old male with 2 episodes of sensory impairments in four extremities and liver dysfunction, developed an acute exacerbation of both sensory impairments and liver dysfunction after administration of interferon-alpha. On admission, neurological examination revealed a mild distal weakness of four extremities, moderate impairment of superficial sensation in hands and severe impairment of deep sensation and areflexia in all extremities. Routine laboratory tests were normal except for a mild liver dysfunction. His serum was positive for antinuclear antibody, but negative for anti-DNA antibody and LE-test. Since he was seropositive for hepatitis B (HB) c antibody but seronegative for HBs antigen and antibody, HBe antigen and antibody, he was considered to be a seroconverted carrier of HB virus. Liver biopsy revealed chronic active hepatitis with marked lymphocytic infiltration. CSF examinations were within normal limits. Sensory conduction studies of median and sural nerves showed no response, but motor conduction studies of median and peroneal nerves were within normal limits. Light and electron microscopic examination of biopsied sural nerve disclosed a moderate decrease in large myelinated fibers, but not in either small myelinated or unmyelinated fibers. Thin-layer chromatography with immunostaining showed the presence of anti-GQ1b antibody in his serum. The anti-GQ1b antibody did not react with GT1a. Oral administration of prednisolone alleviated liver dysfunction, muscle weakness and superficial sensory impairment of four extremities, but not in deep sensation.

Topics: Acute Disease; Adult; Autoantibodies; Autoimmune Diseases; Gangliosides; Hepatitis; Humans; Male; Polyneuropathies; Recurrence; Sensation Disorders