gq1b-ganglioside and Nervous-System-Diseases

Topics: Animals; Autoantibodies; Chronic Disease; Gangliosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Nerve Growth Factors; Nervous System Diseases; Paraproteinemias

Topics: Autoantibodies; Autoimmune Diseases; G(M1) Ganglioside; Gangliosides; Humans; Nervous System Diseases

Autoantibodies to acetylcholine receptors and to voltage-gated calcium and potassium channels are thought to be pathogenic in three peripheral neurological disorders: myasthenia gravis, the Lambert Eaton syndrome and acquired neuromyotonia. However, evidence for the role of antibodies in conditions involving the central nervous system, is scanty or unclear. This review describes the ways in which the roles of autoantibodies have been defined in the peripheral diseases, and discusses the more controversial evidence for involvement of autoantibodies in some central disorders such as multiple sclerosis.

Topics: Autoantibodies; Calcium Channels; Cell Line; Gangliosides; Humans; Isaacs Syndrome; Lambert-Eaton Myasthenic Syndrome; Myasthenia Gravis; Nervous System Diseases; Neurons; Receptors, Cholinergic

Topics: Animals; Enzyme Inhibitors; Gangliosides; Glucosyltransferases; Glycosphingolipids; Morpholines; Nervous System; Nervous System Diseases; Synapses; Synaptic Transmission

Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited.. We conducted a nationwide survey of BBE in the Japanese population in two steps: the first aimed to identify patients with brainstem encephalitis for the specified 3 year period and the second to evaluate whether the clinical picture met our diagnostic criteria for BBE.. The number of patients with brainstem encephalitis was estimated as 704 (95% CI 478 to 930) over the 3 years. The annual onset of BBE was roughly estimated as 100 cases, which accounted for 43% of brainstem encephalitis. BBE was slightly male predominant and often young onset. Among brainstem encephalitis patients, BBE was characterised by antecedent infectious symptoms, oropharyngeal palsy and sensory disturbance at the distal extremities with absent or decreased tendon reflexes, in addition to a triad of symptoms (external ophthalmoplegia, ataxia and impaired level of consciousness) and shorter duration to the peak, with good outcome. Anti-GQ1b antibodies were present in 75% of cases. Several BBE patients with atypical clinical features or without anti-GQ1b antibodies were also identified. These cases often had marked CSF pleocytosis, abnormal brain MRI findings and a longer duration to peak symptoms, sometimes with considerable residual deficits.. BBE is a rare disorder but accounts for a major proportion of brainstem encephalitis. BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery whereas atypical BBE is characterised by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with other possible pathogeneses.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Brain Stem; Child; Child, Preschool; Electroencephalography; Encephalitis; Female; Gangliosides; Health Surveys; Humans; Japan; Magnetic Resonance Imaging; Male; Middle Aged; Nervous System Diseases; Physicians, Primary Care; Surveys and Questionnaires; Treatment Outcome; Young Adult

IgA has an important function in the gastrointestinal immune system. We investigated IgA anti-ganglioside antibodies in Guillain-Barré syndrome (GBS) and Fisher's syndrome (FS) subsequent to Campylobacter jejuni enteritis. In previous studies, serological diagnosis of C. jejuni infection was based on the detection of IgG, IgA, and IgM anti-C. jejuni antibodies. Our study, however, showed that the detection of IgG anti-C. jejuni antibody alone was sufficient for the serological diagnosis of antecedent C. jejuni enteritis in GBS and FS, when the cut-off level was defined for results of sera from C. jejuni-isolated patients. Serological evidence of C. jejuni infection was found in 62 (31%) of 201 GBS patients and 12 (18%) of 65 FS patients. IgA anti-GMI antibody was detected in sera from 33 (16%) of the GBS patients, 1 (2%) of the FS patients, and none of the 46 normal control subjects. IgA anti-GM1 antibody titers were significantly higher in the GBS patients with positive C. jejuni serology than in those with negative serology (P < 0.0001) or the FS patients with positive C. jejuni serology (P = 0.007). IgA anti-GQ1b antibody was detected in sera from 18 (28%) of the FS patients, 9 (4%) of the GBS patients, and none of the normal control subjects. FS patients with positive C. jejuni serology had significantly higher titers of IgA anti-GQ1b antibody than those with negative serology (P = 0.01) or the GBS patients with positive C. jejuni serology (P < 0.0001). We conclude that anti-GM1 and anti-GQ1b IgA antibodies are closely associated with antecedent C. jejuni enteritis in GBS and FS, respectively.

Topics: Antibodies, Bacterial; Antibody Specificity; Autoantibodies; Autoimmune Diseases; Campylobacter Infections; Campylobacter jejuni; Enteritis; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin A; Miller Fisher Syndrome; Nervous System Diseases; Polyradiculoneuropathy

IgM, IgG, IgA, and IgG subclass anti-GM1, anti-GQ1b, and anti-asialo-GM1 (anti-GA1) antibodies, respectively, were investigated by ELISA in serum from neurological and other patients. Increased anti-GM1 occurred mostly in approximately 15-35% of the cases without statistical differences; high percentages were found in Guillain-Barré syndrome (GBS) preceded by gastrointestinal infection and multifocal motor neuropathy. Roughly, IgM anti-GM1 was most frequent; however, distinct IgG and IgA reactions were found i.a. in GBS. A particular IgM anti-mono- and disialoganglioside pattern occurred in a patient with sensorimotor neuropathy and paraproteinemia. Anti-GQ1b was elevated in all Miller-Fisher patients, with some prevalence of IgG2 among IgG subclasses. Cross-reactivity of anti-GQ1b was demonstrated with Campylobacter jejuni lipopolysaccharides. Increased anti-GM1 and/or anti-GA1 was more frequent in systemic lupus erythematosus with central nervous system involvement than without. Incidence of anti-GM1 and anti-GA1 in X-adrenoleukodystrophy was relatively high. Although anti-GSL antibodies seem to have limited diagnostic value, studies of isotypes, subclass patterns, and cross-reactivities may lead to further insight into the origin of (auto) immune responses and their immunepathogenetic role in disease.

Topics: Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; Gangliosides; Gastrointestinal Diseases; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin Isotypes; Immunoglobulin M; Lipopolysaccharides; Nervous System Diseases; Paraproteinemias; Polyradiculoneuropathy

Gangliosides, important constituents of the plasma membrane, are particularly abundant in the nervous system. Some patients develop Guillain-Barré syndrome after the administration of bovine brain gangliosides. We previously showed existence of molecular mimicry between GM1 ganglioside and lipopolysaccharide of Campylobacter jejuni isolated from the patients with Guillain-Barré syndrome, and that between GQ1b and C. jejuni isolated from Fisher's syndrome patients. Moreover, the anti-ganglioside antibody can cause motor nerve dysfunction in vitro. These support the pathogenic significance of anti-ganglioside antibodies. To clarify clinical utility of measurement for anti-GM1 and anti-GQ1b antibodies, we investigated sera from 429 patients with immunoneurological diseases included Fisher's syndrome, Bickerstaff's brainstem encephalitis, acute ophthalmoparesis, Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and controls by enzyme-linked immunosorbent assay. We found very high titers of IgM anti-GM1 antibody in serum from a patient who had been diagnosed as having motor neuron disease. By further electrophysiological study, the patient was diagnosed as having multifocal motor neuropathy. Presence of high IgG anti-GM1 antibody titers was useful for supporting diagnosis of Guillain-Barré syndrome, IgG anti-GQ1b antibody was detected in patients who had paresis of extraocular muscles in Fisher's syndrome, Guillain-Barré syndrome, Bickerstaff's brainstem encephalitis, and acute ophthalmoparesis. This study showed that the measurement for anti-GM1 and anti-GQ1b antibodies are very useful.

Topics: Adolescent; Adult; Autoantibodies; Female; G(M1) Ganglioside; Gangliosides; Humans; Middle Aged; Miller Fisher Syndrome; Nerve Growth Factors; Nervous System Diseases; Polyradiculoneuropathy

Anti-GQ1b antibodies were assayed by an enzyme-linked immunosorbent assay in sera from patients with non-neurological disorders (N = 20), and with various neurological disorders (N = 59), including nine cases of Miller Fisher syndrome, 16 cases of Guillain-Barré syndrome and one case of acute post-infectious ophthalmoparesis. Such antibodies were found in most cases (8 out of 9) of Miller Fisher syndrome, and at very high titres, in one case of Guillain-Barré syndrome characterised by an initial ophthalmoparesis, and in the case of isolated post-infectious ophthalmoparesis. The latter was characterised by a long-lasting occurrence of these antibodies. Anti-GQ1b antibodies are specific for an immune-mediated neuropathy of the cranial, especially oculomotor, nerves.

Topics: Autoantibodies; Autoantigens; Autoimmune Diseases; Campylobacter Infections; Campylobacter jejuni; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Immunoglobulin M; Male; Middle Aged; Miller Fisher Syndrome; Nervous System Diseases; Ophthalmoplegia; Polyradiculoneuropathy; Virus Diseases

Sera from patients with Fisher's syndrome in the acute phase contain immunoglobulin (Ig)G anti-GQ1b ganglioside antibody. Removal of the autoantibody should lead to earlier recovery with less residual neurologic involvement. A tryptophan- or phenylalanin-immobilized polyvinyl alcohol gel column (IM-TR 350 or IM-PH 350) semiselectively adsorbs such autoantibodies as rheumatoid factor, anti-DNA antibody, or anti-acetylcholine receptor antibody. A batchwise adsorption test showed that an IM-TR gel adsorbed a larger amount of the IgG anti-GQ1b antibody than did an IM-PH column. Several patients with Fisher's syndrome therefore were given immunoadsorbent therapy using the IM-TR column without adverse reactions. An ex vivo plasma perfusion study done with the IM-TR column confirmed that it effectively adsorbs the IgG anti-GQ1b antibody. Results of adsorption tests done with various amino acid-immobilized gels suggest that both the hydrophobic force of the side chain and the anionic charge of the carboxylic acid in tryptophan are important in the adsorption of the autoantibody by the IM-TR gel. Immunoadsorption using the IM-TR column, which does not need replacement fluids, offers an alternative type of plasmapheresis for Fisher's syndrome.

Topics: Adolescent; Adult; Aged; Ataxia; Autoantibodies; Autoimmune Diseases; Child; Chromatography, Affinity; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Immunosorbent Techniques; Male; Middle Aged; Nervous System Diseases; Ophthalmoplegia; Plasmapheresis; Polyradiculoneuropathy; Polyvinyl Alcohol; Syndrome; Tryptophan

We report a patient with a relapsing form of the acute sensory neuropathy syndrome associated with IgM-kappa type monoclonal gammopathy of undetermined significance. He rapidly developed marked sensory ataxia without weakness following an upper respiratory tract infection at age 44. The symptoms reached their maximum in a few days, followed by subsequent gradual improvement over a few weeks. However, unsteady gait remained as a chronic deficit. Stepwise progression of his symptoms occurred over 15 years with 10 similar relapses. Sensory nerve conduction studies showed the absence of action potentials, and sural nerve biopsy revealed the marked loss of large myelinated fibers. The patient's serum had an extremely high titer of an IgM monoclonal antibody directed against gangliosides GD2, GD1b, GT1b, and GQ1b.

Topics: Acute Disease; Adult; Carbohydrate Sequence; Gangliosides; Humans; Immunoglobulin M; Male; Molecular Sequence Data; Nervous System Diseases; Recurrence; Sensation