gq1b-ganglioside and Muscle-Weakness

Topics: Action Potentials; Aged; Asymptomatic Diseases; Ataxia; Autoantibodies; Blepharoptosis; Calcium Channels, P-Type; Calcium Channels, Q-Type; Diplopia; Electrodiagnosis; Electromyography; Female; Gangliosides; Humans; Hypesthesia; Lambert-Eaton Myasthenic Syndrome; Median Nerve; Miller Fisher Syndrome; Muscle Weakness; Mydriasis; Neural Conduction; Oculomotor Muscles; Tibial Nerve; Ulnar Nerve

Topics: Bulbar Palsy, Progressive; Facial Paralysis; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Muscle Weakness; Paresthesia

Topics: Antibodies, Anti-Idiotypic; Ataxia; Brain Stem; Cerebellum; Child; Child, Preschool; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Miller Fisher Syndrome; Muscle Weakness; Ophthalmoplegia; Retrospective Studies

Anti-GQ1b antibodies have been found in patients with Miller Fisher syndrome as well as its related conditions. Our aim was to identify the mechanism by which autoantibodies produce various clinical presentations in 'anti-GQ1b antibody syndrome'.. Immunoglobulin G antibodies to ganglioside complex (GSC) of GQ1b or GT1a with GM1, GD1a, GD1b or GT1b were tested in sera from patients with anti-GQ1b (n = 708) or anti-GT1a (n = 696) IgG antibodies. Optical densities of the single anti-GQ1b or anti-GT1a antibodies were used as reference (100%), and those of anti-GSC antibodies were expressed in percentages to reference. The relationships between anti-GSC antibody reactivity and the corresponding clinical features were assessed by multivariate logistic regression analysis.. Ophthalmoplegia and hypersomnolence were significantly associated with complex-attenuated anti-GQ1b and anti-GT1a antibodies. Ataxia was associated with GD1b- and GT1b-enhanced anti-GQ1b antibodies or GM1-enhanced anti-GT1a antibodies. Bulbar palsy was associated with GT1b-enhanced anti-GQ1b antibodies. Neck weakness was associated with GD1a-enhanced anti-GQ1b antibodies. Arm weakness was associated with GD1b-enhanced anti-GQ1b and GD1a-enhanced anti-GT1a antibodies. Leg weakness was associated with GD1a-enhanced anti-GQ1b and anti-GT1a antibodies.. Differences in fine specificity of anti-GQ1b antibodies are associated with clinical features, possibly due to the different expression of gangliosides in different parts of the nervous system.

Topics: Ataxia; Autoantibodies; Bulbar Palsy, Progressive; Disorders of Excessive Somnolence; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Miller Fisher Syndrome; Muscle Weakness; Ophthalmoplegia

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Muscle Weakness; Ophthalmoplegia

A patient with Bickerstaff's brain stem encephalitis (BBE) associated with anti-GQ1b antibody developed coma, severe weakness, and respiratory distress. The patient required ventilatory support. After having failed to improve on steroids, she was treated with plasmapheresis. She improved concomitantly with the plasmapheresis treatment and made a complete recovery. BBE associated with anti-GQ1b antibody is generally considered to be benign, and specific treatments have not been established. The results with this patient suggest that the condition is not always benign, and plasmapheresis may be beneficial in this disorder.

Topics: Adult; Antibodies; Brain Stem; Coma; Electroencephalography; Encephalitis; Evoked Potentials, Somatosensory; Female; Gangliosides; Glucocorticoids; Humans; Immunoglobulin M; Infusions, Intravenous; Methylprednisolone; Muscle Weakness; Nerve Growth Factors; Plasmapheresis; Respiration, Artificial; Respiratory Insufficiency