gq1b-ganglioside and Autoimmune-Diseases

Topics: Antibody Specificity; Autoantibodies; Autoantigens; Autoimmune Diseases; Brain; Carbohydrate Sequence; CD57 Antigens; Chronic Disease; G(M1) Ganglioside; Gangliosides; Globosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunoglobulin M; Molecular Sequence Data; Motor Neuron Disease; Myelin Sheath; Paraproteinemias; Peripheral Nervous System; Polyradiculoneuropathy; Sulfoglycosphingolipids

Some patients develop Guillain-Barré syndrome (GBS) after the administration of bovine gangliosides. Patients with GBS subsequent to Campylobacter jejuni enteritis frequently have IgG antibody to GM1 ganglioside. Miller Fisher syndrome (MFS), a variant of GBS, is associated with IgG antibody to GQ1b ganglioside. Molecular mimicry between GM1 and lipopolysaccharide of C. jejuni isolated from patients with GBS, and between GQ1b and C. jejuni lipopolysaccharides from patients with MFS have been demonstrated. The molecular mimicry between infectious agents and gangliosides may function in the production of anti-ganglioside antibodies. This sugar mimicry is one possible cause of the Guillain-Barré and Miller Fisher syndromes; however, unidentified host factors may contribute to the development of these syndromes.

Topics: Animals; Antigens, Bacterial; Autoantigens; Autoimmune Diseases; Campylobacter Infections; Campylobacter jejuni; Cattle; Epitopes, B-Lymphocyte; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Lipopolysaccharides; Miller Fisher Syndrome; Molecular Mimicry

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

Over the past decade, remarkable progress has been made in our understanding of the pathogenesis of Miller Fisher syndrome (MFS), a clinical variant of Guillain Barré syndrome (GBS). MFS comprises the clinical triad of ataxia, areflexia and ophthalmoplegia. It is associated with acute-phase IgG antibodies to GQ1b and GT1a gangliosides in over 90% of cases which are highly disease specific. Like GBS, MFS is a post-infectious syndrome following diverse infections, but particular attention has been paid to its association with Campylobacter jejuni enteritis. Serostrains of C. jejuni isolated from infected patients bear ganglioside-like epitopes in their lipopolysaccharide core oligosaccharides, which elicit humoral immune responses exhibiting molecular mimicry with GQ1b/GT1a gangliosides. These antibodies are believed to be the principal cause of the syndrome and physiological studies aimed at proving this have focused on the motor-nerve terminal as a potential site of pathogenic action. This review describes these findings and formulates a pathogenesis model based on our current state of knowledge.

Topics: Animals; Autoimmune Diseases; Chromatography, Thin Layer; Gangliosides; Humans; Infections; Miller Fisher Syndrome; Oligosaccharides; Peripheral Nerves; Phenotype; Rats

Graves' disease and anti-GQ1b antibody syndrome are both autoimmune diseases, and there have been few reports on whether there is a correlation between the two. In this study, we present the case of a woman who was diagnosed with Graves' disease and anti-GQ1b antibody syndrome in succession.. The chief complaints of this patient were limb weakness and blurred vision. Graves' disease was diagnosed by examination of thyroid function and thyroid autoantibodies, but the clinical symptoms were not relieved after antihyperthyroidism treatment. Finally, it was found that Graves' disease was complicated by anti-GQ1b antibody syndrome, and the symptoms were relieved after treatment with glucocorticoids and intravenous immunoglobulin. We also explored the possible mechanism of these diseases through a literature review.. We report a rare case of the cooccurrence of Graves' disease and anti-GQ1b antibody syndrome. Immune dysregulation might be the pathogenesis of the association, but there is no precise supporting evidence, and more research is needed.

Topics: Autoantibodies; Autoimmune Diseases; Female; Gangliosides; Graves Disease; Humans

Anti-GQ1b antibodies can be detected in the serum of patients with Miller Fisher syndrome (MFS) and its incomplete forms such as acute ophthalmoparesis (AO), acute ptosis, acute mydriasis, acute oropharyngeal palsy and acute ataxic neuropathy (AAN), as well as in pharyngeal-cervical-brachial weakness, Bickerstaff brainstem encephalitis (BBE) and in overlap syndromes with Guillain-Barré syndrome (MFS-GBS, BBE-GBS). We searched the laboratory medicine database at University Hospitals Leuven between 2002 and 2017 for serum samples with anti-GQ1b IgG antibodies. We identified eight patients with anti-GQ1b antibodies: 4 MFS, 2 AO, 1 MFS-GBS and 1 AAN. Mean age was 57 years and five patients were males. Preceding illness was present in all patients. At nadir, we observed most frequently gait disturbance, external ophthalmoplegia and absent/decreased reflexes. Albumino-cytological dissociation was present in four patients. Mean time between onset and nadir was 4 days, between onset and recovery 2.5 months. Five patients recovered completely and three had minor residual symptoms. Interestingly, one patient with AO experienced a second identical episode, approximately 1 year after the first one. Our data confirm the broad clinical spectrum associated with the presence of anti-GQ1b IgG antibodies. Incomplete MFS subtypes such as AO are a challenge for diagnosis, because of the limited (though invalidating) clinical presentation and the lack of confirming ancillary tests. Subacute onset of ophthalmoplegia and/or ataxia should urge the clinician to include the anti-GQ1b antibody syndrome in the differential diagnosis.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Autoantigens; Autoimmune Diseases; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia; Paraplegia; Syndrome; Young Adult

Topics: Adult; Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases; Brain Stem; Cognitive Dysfunction; Diplopia; Encephalitis; Female; Gangliosides; Humans

We report the first case of Listeria monocytogenes meningoencephalitis associated with anti-GQ1b antibody syndrome in an immunocompetent adult. A prompt diagnosis, made thanks to the multidisciplinary contribution, allowed a combined therapeutic approach leading to final favourable outcome, despite several intercurrent complications.

Topics: Autoantibodies; Autoimmune Diseases; Encephalitis; Gangliosides; Humans; Male; Meningitis, Listeria; Middle Aged; Miller Fisher Syndrome

Antiganglioside antibodies are found in various neurological disorders that constitute a continuum from peripheral neuropathy to encephalitis. However, nystagmus has rarely been described in patients with ataxia associated with antiganglioside antibodies.. From January 2008 to July 2009, we identified 3 patients with acute ataxia and nystagmus in 2 University Hospitals of Korea, who were found to have anti-GD1b, anti-GM1, or anti-GQ1b antibodies.. In addition to acute ataxia, all 3 patients showed various combinations of nystagmus, which included central positional nystagmus (n = 3), vertical nystagmus (n = 1), and periodic alternating nystagmus (n = 1). The spontaneous and positional nystagmus were mostly detectable only with the elimination of fixation and magnification of the eyes using video goggles. Two patients also exhibited gaze-evoked nystagmus that was noticeable without the aid of video goggles. Patients had serum IgG antibodies to GD1b, GM1, or GQ1b. Cerebrospinal fluid examination, nerve conduction studies, and brain MRI were normal. In all patients, the symptoms and signs resolved over 3-12 months.. Various forms of nystagmus with acute ataxia may be a sole or predominant manifestation of disorders related to antiganglioside antibodies. The nystagmus indicates a central pathology involving the cerebellum or brainstem in this antibody-associated disorder. Antiganglioside antibodies should be measured in patients with nystagmus and acute ataxia of undetermined etiology.

Topics: Acute Disease; Adolescent; Adult; Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases; Cerebrospinal Fluid; Dizziness; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Humans; Immunoglobulin G; Male; Nystagmus, Pathologic; Postural Balance; Sensation Disorders; Vertigo

To prospectively study anti-GQ1b antibody positive cases of acute ophthalmoparesis (AO) clinically and electrophysiologically.. Nine consecutive cases presenting with predominantly acute ophthalmoplegia were assessed clinically and had stimulated single fibre electromyography (SFEMG) of the orbicularis oculi at presentation. All had magnetic resonance imaging brain scans and anti-GQ1b antibody titres determined.. Four cases had elevated anti-GQ1b antibody titres and abnormal SFEMG studies, which improved in tandem with clinical recovery over three months. Five other anti-GQ1b antibody negative cases were diagnosed as diabetic related cranial neuropathy, idiopathic cranial neuropathy, ocular myasthenia gravis, and Tolosa-Hunt syndrome. All five cases showed complete recovery over a three month period.. This study demonstrated electrophysiologically the dynamic improvement of neuromuscular transmission of anti-GQ1b antibody positive cases of AO, in tandem with clinical recovery. SFEMG is of value in differentiating weakness due to neuromuscular transmission defect from neuropathy in these clinical situations.

Topics: Acute Disease; Autoantibodies; Autoimmune Diseases; Electromyography; Electrophysiology; Gangliosides; Humans; Male; Middle Aged; Ophthalmoplegia; Prospective Studies

To examine the clinical features of acute ophthalmoparesis (AO) (without ataxia) associated with anti-GQ1b immunoglobulin G (IgG) antibody.. Retrospective observational case series.. Twenty-one subjects with AO (without ataxia) who had anti-GQ1b IgG.. Clinical features of 21 subjects with AO were analyzed.. Seventeen had symptoms of antecedent infection. Gaze limitation was bilateral in 16 subjects and unilateral in five, indicative that laterality does not always negate AO. Nine subjects showed abducens paresis, and two limitation of abduction and adduction. Eight, who initially had bilateral abducens palsy, subsequently had impairment of adduction and vertical movement. These showed that bilateral abducens palsy followed by oculomotor nerve involvement is characteristic of AO. Muscle stretch reflexes were normal in nine subjects, hypoactive in eight, absent in three, and brisk in one. Distal paresthesias were present in seven subjects. Acellular cerebrospinal fluid (CSF) associated with raised protein concentration was detected in three.. Antecedent infectious symptoms, characteristic limitation of ocular movement, areflexia, distal paresthesias, and CSF albuminocytologic dissociation are useful markers for diagnosing AO as well as anti-GQ1b IgG. AO can be considered a mild form of Miller Fisher syndrome or a regional variant of Guillain-Barré syndrome.

Topics: Abducens Nerve Diseases; Acute Disease; Adolescent; Adult; Aged; Autoantibodies; Autoimmune Diseases; Campylobacter Infections; Campylobacter jejuni; Child; Diplopia; Female; Gangliosides; Gastrointestinal Diseases; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia; Paresthesia; Retrospective Studies

To describe the effects of immunoadsorption therapy with a tryptophan-immobilized column on Fisher syndrome associated with IgG anti-GQ1b ganglioside antibody.. Three patients with Fisher syndrome and with a high serum IgG anti-GQ1b antibody titer underwent four to nine sessions of immunoadsorption therapy with a tryptophan-immobilized column. Using enzyme-linked immunosorbent assay (ELISA), we determined the differences in IgG anti-GQ1b antibody titers.. ELISA disclosed that the IgG anti-GQ1b antibody titers of the serum samples collected from the inlet of the column were markedly higher than those collected from the outlet for all three patients. Moreover, after completion of the immunoadsorption therapy, the patients' serum IgG anti-GQ1b antibody titers were markedly lower than they were before the immunoadsorption therapy. The patients' ophthalmoparesis decreased in severity during the therapy.. These findings suggest that immunoadsorption therapy with the tryptophan-immobilized column is an effective method for removing IgG anti-GQ1b antibody from serum.

Topics: Adolescent; Adult; Autoantibodies; Autoimmune Diseases; Chromatography, Affinity; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Immunosorbent Techniques; Male; Miller Fisher Syndrome; Tryptophan

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

Serum antibody against ganglioside GQ1b is reported to be closely associated with immune mediated ophthalmoplegia in the Fisher and Guillain-Barré syndromes. Its presence against glycolipids, in particular ganglioside GQ1b, was investigated in patients with ophthalmoplegia of unknown origin.. 16 patients with ophthalmoplegia, the cause of which could not be confirmed from clinical findings or diagnostic testing, were tested. 34 patients who had ophthalmoplegia of definite cause, 16 healthy people, and 23 patients with typical Fisher syndrome served as the controls. The ELISA was used to check for serum antibodies against glycolipids in all study participants.. Two of the 16 patients with ophthalmoplegia of unknown cause had serum IgG antibody against GQ1b but not against other glycolipids, and 22 of the 23 patients with typical Fisher syndrome had this antibody. No anti-GQ1b antibodies were found in the patients with ophthalmoplegia of definite cause or in the normal controls.. A common underlying cause appears to bring about the pathogenesis of palsy in Fisher syndrome and in the ophthalmoplegia with positive anti-GQ1b IgG antibody, called atypical Fisher syndrome. This antibody may prove a useful clinical marker for differentiating Fisher syndrome, typical and atypical, in patients with ophthalmoplegia.

Topics: Adult; Aged; Autoantibodies; Autoimmune Diseases; Biomarkers; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Nerve Growth Factors; Ophthalmoplegia

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

Topics: Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases; Ganglia, Sensory; Gangliosides; Glycolipids; Humans; Peripheral Nervous System 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 investigated serological human leukocyte antigen (HLA) types in patients with histories of Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with ophthalmoplegia, in whom serum anti-GQ1b IgG antibody was present during the acute phase. We examined class I antigens (A, B and C) in 32 patients and class II antigens (DR and DQ) in 30, but found no association. We conclude that particular serologically defined HLA types are not preferred for the immunoresponse of anti-GQ1b IgG antibody in MFS and GBS.

Topics: Autoantibodies; Autoantigens; Autoimmune Diseases; Gangliosides; HLA Antigens; Humans; Immunoglobulin G; Polyradiculoneuropathy

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

We studied serum anti-glycolipid antibodies by enzyme-linked immunosorbent assay and thin-layer chromatography-enzyme immunoassay in six consecutive patients with typical Miller Fisher syndrome. In all six, increased activity of IgG antibody against ganglioside GQ1b was present in the early phase and reduced with time, whereas such activity was not detected in normal control subjects and disease control subjects including those with Guillain-Barré syndrome. Anti-GQ1b IgG antibody is a new possible diagnostic marker of Miller Fisher syndrome and could well be related to the disease process itself.

Topics: Adult; Aged; Antibody Specificity; Ataxia; Autoantibodies; Autoimmune Diseases; Biomarkers; Female; Gangliosides; Glycolipids; Humans; Immunoglobulin G; Male; Middle Aged; Multiple Sclerosis; Ophthalmoplegia; Polyradiculoneuropathy; Reflex, Abnormal; Syndrome