gq1b-ganglioside and Guillain-Barre-Syndrome

Fisher syndrome has been regarded as a peculiar inflammatory neuropathy with ophthalmoplegia, ataxia, and areflexia, whereas Bickerstaff brainstem encephalitis has been considered a pure central nervous system disease characterized by ophthalmoplegia, ataxia, and consciousness disturbance. Both disorders share common features including preceding infection, albumin-cytological dissociation, and association with Guillain-Barré syndrome. The discovery of anti-GQ1b IgG antibodies further supports the view that the two disorders represent a single disease spectrum. The lesions in Fisher syndrome and Bickerstaff brainstem encephalitis are presumably determined by the expression of ganglioside GQ1b in the human peripheral and central nervous systems. Bickerstaff brainstem encephalitis is likely to represent a variant of Fisher syndrome with central nervous system involvement.

Topics: Animals; Autoantibodies; Brain Stem; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome

Bickerstaff brainstem encephalitis (BBE) is characterized by acutely progressive bilateral ophthalmoparesis and ataxia with impaired consciousness or pyramidal signs, or both; all of which are followed by a monophasic course with good recovery. Alike Guillain-Barré syndrome (GBS), BBE is proposed to have an autoimmune mechanism triggered by antecedent infection. The nationwide epidemiologic survey for BBE, which the author had performed in Japan, suggests that 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 characterized by delayed recovery, negative anti-GQ1b antibodies, and abnormal cerebrospinal fluid and brain MRI findings with other possible pathogeneses.

Topics: Age Factors; Autoantibodies; Autoimmunity; Biomarkers; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Japan; Male; Miller Fisher Syndrome; Sex Factors

The discovery of the association of the anti-GQ1b IgG antibody with the postinfectious clinical syndromes of ophthalmoplegia, ataxia, and areflexia helped house the phenotypes of the Miller Fisher syndrome (MFS), atypical MFS, Guillain-Barré syndrome with ophthalmoplegia, and Bickerstaff's brainstem encephalitis under one roof. The neuro-ophthalmologic signs classically predominate and may vary from case to case, but they maintain clinically recognizable patterns that assist with the diagnosis. The identification of a common lipopolysaccharide on the plasma membrane in human cranial and peripheral nerves at the GQ1b epitope and on infectious particles of bacteria and viruses (ie, Campylobacter jejuni) demonstrates molecular mimicry. The high frequency of oculomotor dysfunction is partially explained by the tissue ganglioside concentration and distribution and the attraction of antibody-stimulating complement activation. Current experimental treatment targets antibody removal and neutralization and prevents membrane attack complex formation through deactivation of complement. This article aims to bring together the historically disparate opinions on the origins of these syndromes as either a purely peripheral nervous system or central nervous system dysfunction, highlight the clinical neuro-ophthalmologic signs, discuss some of the biology of the anti-GQ1b antibody, and review imaging abnormalities and treatment of this fascinating disorder.

Topics: Diagnosis, Differential; Gangliosides; Guillain-Barre Syndrome; Humans; Immunotherapy; Magnetic Resonance Imaging; Miller Fisher Syndrome; Ophthalmoplegia; Peripheral Nerves

Although acute inflammatory polyneuropathy (AIP) and immune thrombocytopenic purpura (ITP) are both believed to be immune-mediated disorders, only a few cases have been reported in which these two diseases co-existed. We describe a case of a 67-year-old patient who developed quadriparesis, ophthalmoplegia and severe sensory impairment along with thrombocytopenia. Detailed examinations, including the measurement of anti-ganglioside antibodies and anti-glycoprotein-IIb-IIIa-IgG-producing B-cells, revealed that he developed AIP and ITP. By reviewing past similar reports, we noticed that AIP associated with ITP tends to manifest severe sensory impairment and is often preceded by upper respiratory tract infection, but not by gastrointestinal infection.

Topics: Acute Disease; Aged; Autoantibodies; Gangliosides; Glucocorticoids; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Male; Pharyngitis; Platelet Count; Prednisone; Purpura, Thrombocytopenic, Idiopathic

Topics: Antibody-Dependent Cell Cytotoxicity; Autoantibodies; Campylobacter jejuni; Complement Activation; Gangliosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunity, Cellular; Immunoglobulin G; Immunoglobulins, Intravenous; Plasmapheresis; Prognosis; T-Lymphocytes

Topics: Autoantibodies; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Ophthalmoplegia; Reference Values; Specimen Handling

Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia. Ninety-two per cent of the patients involved had had an antecedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and generally a good outcome. Serum anti-GQ1b IgG antibody was positive in 66%, and MRI showed brain abnormality in 30% of the patients. Another striking feature was the association with flaccid symmetrical tetraparesis, seen in 60% of the patients. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Limb weakness in the BBE cases studied was considered the result of overlap with the axonal subtype of Guillain-Barré syndrome. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. A considerable number of BBE patients have associated axonal Guillain-Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Ataxia; Autoantibodies; Brain Stem; Chi-Square Distribution; Child; Child, Preschool; Electroencephalography; Electromyography; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Male; Middle Aged; Motor Neurons; Ophthalmoplegia; Quadriplegia

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.

Topics: Autoantibodies; Brain Stem; Child; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Miller Fisher Syndrome

Guillain Barré syndrome is one of the best examples of a post infectious immune disease and offers insights into the mechanism of tissue damage in other more common autoimmune diseases. Controlled epidemiological studies have linked it to infection with Campylobacter jejuni in addition to other viruses including cytomegalovirus and Epstein Barr virus. The syndrome includes several pathological subtypes, of which the most common is a multifocal demyelinating disorder of the peripheral nerves in close association with macrophages. Evidence from histological examination of peripheral nerve biopsy and postmortem samples suggests that both cell mediated and humoral mechanisms are involved in the pathogenesis. Immunological studies suggest that at least one third of patients have antibodies against nerve gangliosides, which in some cases also react with constituents of the liposaccharide of C jejuni. In the Miller Fisher variant of the disease, these antiganglioside antibodies have been shown to produce neuromuscular block, and may in part explain the clinical signs of that disorder. Treatment with both intravenous immunoglobulin and plasma exchange reduces the time taken for recovery to occur, although mortality remains around 8%, with about 20% of patients remaining disabled.

Topics: Autoantibodies; Axons; Bacterial Infections; Electrophysiology; Female; Gangliosides; Glucocorticoids; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Lymphocytes; Macrophages; Male; Neuromuscular Junction; Plasma Exchange; Prognosis

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

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; Biomarkers; Campylobacter jejuni; Cytomegalovirus; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Immunoglobulins, Intravenous; Plasma Exchange; Prognosis

Miller-Fisher syndrome (MFS) together with Guillan-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) are considered to form a continuous clinical spectrum of the same disease, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. The frequency of overlapping clinical signs and the risk of recurrence are independent and very low, but no cases of GQ1b-seropositive recurrent MFS overlapping with GBS and BBE have been described so far. Here, we describe for the first time an atypical case of recurrent GQ1b-seropositive MFS overlapping GBS and BBE, 12 years after a previous GQ1b-seronegative typical MFS episode. Our case expands the clinical spectrum of recurrent MFS, and it should prompt clinicians to investigate the presence of anti-ganglioside antibodies in recurrent MFS even when these were negative in the previous episode, especially in those presenting with overlapping spectrum symptoms and a critically ill picture during the second episode.

Topics: Adult; Autoantibodies; Autoantigens; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Recurrence

Antibodies against ganglioside complexes (GSCs) are associated with various clinical features and subtypes of Guillain-Barré syndrome (GBS).. One-hundred patients were evaluated for antibodies to GSCs formed by combination of GM1, GM2, GD1a, GD1b, GT1b, and GQ1b using manual enzyme linked immuno-sorbent assay (ELISA).. Twenty-six patients were GSC antibody-positive, most frequent being against GM1-containing GSC (76.9%). Gender distribution, mean age, symptom-duration, antecedent events, electrophysiological subtypes, requirement for mechanical ventilation, and median duration of hospital stay were comparable between the GSC antibody-positive and negative groups. There was no association between specific GSC antibody and electrophysiological subtypes or clinical variants. After controlling for false discovery rate (FDR) using the Benjamini-Hochberg method, the number of subjects who improved in overall disability sum score, modified Erasmus GBS outcome score, and neuropathy symptom score at discharge was significantly higher in the GSC antibody-positive group. Improvements in Medical Research Council sum scores and Hughes Disability Scale during the hospital stay between the GSC antibody-positive and negative groups were not significantly different after controlling for FDR.. The GSC antibody-positive group had better outcome at hospital discharge in some of the disability scores. Pathophysiological pathways among patients without GSC antibodies may be different and this requires further evaluation.

Topics: Adolescent; Adult; Autoantibodies; Case-Control Studies; Cohort Studies; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; G(M2) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Immunologic Factors; India; Male; Middle Aged; Plasmapheresis; Respiration, Artificial; Time Factors; Treatment Outcome; Young Adult

Approximately 15%-20% of patients with Guillain-Barré syndrome (GBS) are unable to walk independently at 6 months from the onset of neurological symptom. The modified Erasmus GBS outcome score (mEGOS) has been reported as a prognostic tool.Herein we investigated the association between a poor outcome, inability to walk independently at 6 months and presence of antiganglioside antibodies.. The clinical and serological data of 177 patients with GBS were retrospectively collected in Japan to assess the associations between a poor outcome and serum IgG antibodies against each ganglioside (GM1, GD1a, GalNAc-GD1a, GQ1b and GT1a). In addition, we investigated whether the combination of mEGOS and serum IgG antibodies against gangliosides is useful in predicting a poor outcome.. The patients with IgG anti-GD1a antibodies more frequently showed poor outcomes than those without these antibodies (9 (36%) of 25 vs 8 (6%) of 127 patients, p<0.001). Particularly, 80% showed a poor outcome when they had both serum IgG anti-GD1a antibody and a high mEGOS of ≥10 on day 7 of admission.. The combination of serum IgG anti-GD1a antibodies and a high mEGOS could help in making a more accurate prognosis of patients than mEGOS alone, especially for predicting poor outcomes.

Topics: Age Factors; Autoantibodies; Diarrhea; Electrodiagnosis; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Mobility Limitation; Prognosis; Respiration, Artificial; Retrospective Studies

A 60-year-old woman with a 3-day history of ataxic gait, blurred vision, and upper extremity paresthesia was admitted to our hospital. She presented with severe visual disturbances (finger counting), ophthalmoplegia, neck weakness, and sensory ataxia. Serum anti-GQ1b antibody, anti-GM3 antibody, and anti-GD3 antibody were strongly positive, which might contribute to the pathogenesis. Since we suspected Guillain-Barré syndrome (GBS), intravenous immunoglobulin therapy (IVIg) and high-dose steroid therapy were administered; however, improvements in her visual acuity were minimal. Additional IVIg and high-dose steroid therapy resulted in limited visual acuity improvements. Therapeutic strategies for patients with GBS and refractory optic neuropathy remain controversial.

Topics: Autoantibodies; Biomarkers; Female; G(M3) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Methylprednisolone; Middle Aged; Optic Nerve Diseases; Pulse Therapy, Drug

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

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

Periodic alternating ping-pong gaze (PPG) is a rare disease with few reports. To our knowledge, there was no report on anti GQ1b antibody syndrome accompanied by PPG. This paper reported a case of anti GQ1b antibody syndrome with Bickerstaff's Encephalitis (BBE) overlapping classic Guillain-Barre Syndrome (GBS) after aortic valve replacement, accompanied by an excessive PPG in the course of diagnosis and treatment, this was indeed rarely.. A 55-year-old male patient was admitted to our hospital with intermittent chest tightness for 3 months, and his condition has worsened in the past 10 days. Aortic valve replacement was performed because of the existence of the moderate and severe stenosis of aortic valve. Horizontal movement of the eyeball was involuntarily slow. The eyeball hovered and returned from one side to the other horizontally for 3-4 s per cycle. In combination with the patient's typical clinical and laboratory tests, the final diagnosis was anti GQ1b antibody syndrome BBE combined with GBS, accompanied by saccadic ping pong gaze. Intravenous immunoglobulin (0.4 g/kg) was given for immunomodulation, methylprednisolone (1000 mg) therapy and symptomatic treatment were performed in the patient.. The patients were discharged from hospital on the thirtieth day because of economic reasons. After 6 months of follow up, the patients left behind a lack of fluency in speech and limb mobility, but the basic life can be taken care of by himself.

Topics: Aortic Valve Stenosis; Autoantibodies; Cardiovascular Surgical Procedures; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Heart Valve Prosthesis; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Ocular Motility Disorders

Anti-glycolipid antibodies are key to revealing the pathomechanisms of Guillain-Barré syndrome (GBS). There are correlations between the antigen specificities of the antibodies, clinical features, and preceding infectious agents. It has also been found that some glycoantigens are localized in human peripheral nervous tissues, corresponding to the clinical features. Antibody-detection methods are still evolving. The discovery of antibodies against glycolipid complexes expanded the horizon of anti-glycolipid research in GBS, which had started from isolated antigens. Recently, IgG antibodies against ganglioside GQ1b-related antigens that required Ca

Topics: Autoantibodies; Calcium; Gangliosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Miller Fisher Syndrome

Antibodies to the ganglioside GD1b have been reported in various forms of immune-mediated neuropathy, but their clinical relevance for diagnosis and prognosis is unknown. We investigated the prevalence of anti-GD1b antibodies in acute and chronic immune-mediated neuropathies, and the clinical presentation and outcome in Guillain-Barré syndrome (GBS) and Miller Fisher-GBS overlap syndrome (MF-GBS). Anti-GD1b, anti-GM1 and anti-GQ1b antibodies were tested in serum of patients with GBS (N = 165), Miller Fisher syndrome (N = 10), MF-GBS (N = 28), monoclonal gammopathy of unknown significance neuropathy (MGUS; N = 101), chronic inflammatory demyelinating polyneuropathy (N = 29), paraneoplastic syndrome with anti-Hu-associated neuropathy (PNS; N = 11), other auto-immune diseases (AID; N = 60), and healthy controls (HC; N = 60). All samples were tested by enzyme-linked immunosorbent assay according to the Inflammatory Neuropathy Cause and Treatment protocol. IgM anti-GD1b antibodies were found in GBS (N = 4; 2.4%), MGUS (N = 3; 3.0%), and PNS patients (N = 1; 9.1%). IgG anti-GD1b antibodies were found in GBS (N = 20; 12.1%) and MF-GBS (N = 4; 14.3%) patients, but not in the AID and HC group. In the combined group of MF-GBS and GBS patients ((MF-)GBS), 14/36 (38.9%) patients with IgG anti-GD1b antibodies also had IgG anti-GM1 antibodies, and IgG anti-GD1b and IgG anti-GQ1b antibodies were found in 3/29 (10.3%) patients. Patients with (MF-)GBS and anti-GD1b without anti-GM1 antibodies did not differ regarding sensory disturbances or disease severity but recovered faster regarding the ability to walk independently compared with patients without anti-GD1b antibodies (P = 0.031) and with patients with both anti-GD1b and anti-GM1 antibodies (P = 0.034). In conclusion, testing for anti-GD1b antibodies may identify a specific group of immune-mediated neuropathies and (MF-)GBS patients with only anti-GD1b antibodies tend to recover faster.

Topics: Adult; Aged; Autoantibodies; Autoantigens; Autoimmune Diseases of the Nervous System; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Lupus Erythematosus, Systemic; Middle Aged; Miller Fisher Syndrome; Monoclonal Gammopathy of Undetermined Significance; Paraneoplastic Polyneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Sjogren's Syndrome

Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome (GBS) with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty-three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS-GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS-GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS-GBS overlap syndrome. No early predictors for progression from MFS to MFS-GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.

Topics: Adult; Autoantibodies; Consciousness Disorders; Disease Progression; Female; Follow-Up Studies; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia; Outcome Assessment, Health Care; Prognosis; Risk Factors

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

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

Guillain-Barré Syndrome (GBS) is classified into the two major subtypes; acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Previous studies have suggested that AIDP is predominant and AMAN is rare in Western countries, whereas AMAN is not always uncommon in East Asia. We aimed to clarify the incidence of the subtypes of GBS in Japan.. We performed a prospective multicentre survey over 3 years (2007-2010). Clinical and electrophysiological findings were collected from 184 patients with GBS in 23 tertiary neurology institutes. Anti-ganglioside antibodies were measured by ELISA. We also surveyed the incidence of Fisher syndrome (FS).. By electrodiagnostic criteria of Ho et al, patients were classified as having AIDP (40%), or AMAN (22%), or unclassified (38%). Anti-GM1 IgG antibodies were found for 47% of AMAN patients, and 18% of AIDP patients (p<0.001). There were no specific regional trends of the electrodiagnosis and anti-GM1 positivity. During the same study period, 79 patients with FS were identified; the percentage of FS cases out of all cases (FS/(GBS+FS)) was 26%.. The frequency of GBS patients with the electrodiagnosis of AMAN by single nerve conduction studies is approximately 20% in Japan, and the AMAN pattern is closely associated with anti-GM1 antibodies. The incidence of FS appears to be much higher in Japan than in Western countries.

Topics: Electrodiagnosis; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Incidence; Japan; Male; Middle Aged; Miller Fisher Syndrome; Motor Neurons; Neural Conduction; Prospective Studies; Symptom Assessment

Plasma exchange and intravenous immunoglobulin are effective in treating Guillain-Barré syndrome (GBS) probably because the former removes IgG autoantibodies and complement and the latter inhibits complement activation subsequent to the autoantibody binding to peripheral nerve antigens. IgG degrading enzyme of Streptococcus pyogenes (IdeS) can cleave the pathogenic autoantibodies into F(ab')2 and Fc. The purpose of this study is to show whether IdeS has novel therapeutic potential for GBS. Sera with anti-ganglioside IgG antibodies from 15 patients with GBS or Miller Fisher syndrome were used. We tested whether IdeS cleaved the anti-ganglioside IgG antibodies and inhibited deposition of activated complement component on ELISA plates. IdeS efficiently cleaved IgG and blocked complement activation mediated by anti-GM1, anti-GD1a and anti-GQ1b IgG antibodies. IdeS has therapeutic potential for GBS and related conditions.

Topics: Autoantibodies; Bacterial Proteins; Complement Activation; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Protein Binding

This is the case of a 79-year-old man with chronic lymphocytic leukemia who presented with Guillain-Barré syndrome with features overlapping with the Miller Fisher syndrome and Bickerstaff brainstem encephalitis and positive antiganglioside GQ1b antibody about 6 months after treatment with bendamustine and rituximab. His clinical and neurologic condition continued to deteriorate despite sequential treatment with corticosteroids, intravenous immunoglobulin and plasmapheresis, but in the end, he had a complete and durable response to treatment with alemtuzumab.

Topics: Aged; Alemtuzumab; Antibodies, Monoclonal, Humanized; Antibodies, Monoclonal, Murine-Derived; Antigens, CD; Antigens, Neoplasm; Antineoplastic Combined Chemotherapy Protocols; Autoantibodies; Autoantigens; Bendamustine Hydrochloride; CD52 Antigen; Combined Modality Therapy; Consciousness Disorders; Gangliosides; Glycoproteins; Guillain-Barre Syndrome; Herpes Zoster; Herpesvirus 3, Human; Humans; Immunoglobulins, Intravenous; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Methylprednisolone; Miller Fisher Syndrome; Nitrogen Mustard Compounds; Plasmapheresis; Remission Induction; Rituximab; Virus Activation

Recurrence of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS) is uncommon. We retrospectively studied the cases of 93 consecutive patients with GBS and FS who were admitted to our hospital between January 2000 and March 2013. We analyzed the clinical features of and anti- glycolipid antibodies in patients who experienced recurrence. Of the 93 patients, 53, 37, and 3 had GBS, FS, and overlapping GBS and FS, respectively. There were 6 recurrences in 4 patients, all of whom were women; their onset age ranged from 26 to 51 years, and the average time to recurrence ranged from 9 months to 25 years. The recurrence rate of FS was 10.8%. On the recurrence, 2 patients showed FS (5.4%) and 2 patients showed overlap of GBS and FS (5.4%). All patients with recurrence showed good prognosis and increased anti-GQ1b glycolipid antibody levels both at the initial episode and at recurrence. Immunological examinations, including those for detecting changes in anti-glycolipid antibodies, are important for clarifying the pathomechanism of recurrence in GBS and FS.

Topics: Adult; Age of Onset; Aged; Autoantibodies; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Recurrence; Retrospective Studies; Time Factors

We report a case of a 23-year-old man diagnosed with overlapping Bickerstaff brainstem encephalitis (BBE) and Guillain-Barre syndrome (GBS). The patient initially presented with fever and headache and gradually developed ataxia, disturbance of consciousness, respiratory muscle paralysis, bilateral facial paralysis and quadriplegia accompanied by significant atrophy of limb, temporalis and masseter muscles. Brain MRI revealed abnormality in the left basal ganglia, thalamus, and rightside posterior limb of the internal capsule. Electromyogram indicated neurogenic damage (mainly axonal damage) in the upper and lower limbs and bilateral facial nerve damage. Cerebrospinal fluid (CSF) collected via lumbar puncture was colorless and transparent with a pressure of 330 mm H2O. The white blood cell count in CSF was 200×106/L, the protein concentration was 1.25 g/L, and Pandy's reaction was positive. Both the blood and CSF were negative for GQ1b antibody. The patient was clinically diagnosed with overlapping BBE and GBS. After treatment with ventilator assisted breathing, hormone therapy, neurotrophic and anti-infection therapies, and symptomatic and supportive care for more than three months, spontaneous breathing was restored. By the 5-month follow-up examination, the patient had completely recovered and returned to work. Like GBS and Fisher syndrome, BBE might be an anti-GQlb IgG antibody syndrome. Although the serum GQlb IgG antibody-positive rate for BBE is only 66%, a normal brainstem MRI or GQlb lgG antibody-negative finding cannot completely rule out BBE. Therefore, identifying critical illness polyneuropathy for patients with respiratory muscle paralysis and tracheal extubation difficulties at early stages is clinically important.

Topics: Autoantibodies; Brain Stem; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Magnetic Resonance Imaging; Male; Tomography, X-Ray Computed; Young Adult

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bicker staff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

Topics: Adolescent; Adult; Aged; Antibodies, Anti-Idiotypic; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

A 29-year-old female developed diplopia, nasal voice and gait disturbance after an upper respiratory infection. On admission, she presented with bilateral external ophthalmoplegia, slight bilateral facial nerve palsy, dysarthria, dysphagia, cervical and brachial muscle weakness, ataxia and areflexia. She had serum anti-GT1a, anti-GQ1b and anti-galactocerebroside IgG antibodies. She was diagnosed with an overlap case of Fisher syndrome and pharyngeal-cervical-brachial variant of Guillain-Barré syndrome. Intravenous immunoglobulin therapy was effective for the ophthalmoplegia and ataxia, but did not improve the bilateral facial nerve palsy and brachial muscle weakness. The facial nerve palsy clearly worsened despite improvement in other symptoms, and therefore high-dose intravenous methylprednisolone therapy was added. The distinct response to treatment may be caused by different activity, production, clearance and reactivity to intravenous immunoglobulin of the autoantibodies. The present case suggests that treatment response and patterns of recovery differ according to the causative anti-ganglioside antibodies.

Topics: Adult; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Methylprednisolone; Miller Fisher Syndrome; Myosin Light Chains; Proteins; Pulse Therapy, Drug; Treatment Outcome

We report a patient with a Guillain-Barré syndrome (GBS) revealed by a posterior reversible encephalopathy syndrome (PRES). The PRES is typically associated with bilateral parieto-occipital T2 and FLAIR hyperintense MRI lesions and observed in various etiologic conditions leading to acute arterial hypertension. PRES results from a breakdown of the circulatory autoregulation, many in the posterior cerebral territories. GBS can be considered as an independent risk factor of PRES, due to acute dysautonomia and pain with consecutive arterial hypertension, as well as to cytokine production changing capillary permability. Such patients with PRES-revealed GBS may be treated with intravenous immunoglobulin therapy only after exclusion of any ischemic or hemorrhagic cerebral complications, and after control of the blood pressure and of the encephalopathic signs and symptoms.

Topics: Aged; Confusion; Electromyography; Female; Gangliosides; Guillain-Barre Syndrome; Homeostasis; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Neurologic Examination; Occipital Lobe; Pain; Parietal Lobe; Posterior Leukoencephalopathy Syndrome; Primary Dysautonomias

IgG anti-GQ1b antibodies are associated with Fisher syndrome (FS), Bickerstaff brainstem encephalitis (BBE), acute ophthalmoparesis and overlap of FS or BBE with Guillain--Barré syndrome (GBS) (FS/GBS or BBE/GBS). It has not been clearly established if the primary pathology of these disorders is demyelinating or axonal in nature. Rapid resolution of conduction slowing or block without signs of demyelination--remyelination has been reported in axonal subtypes of GBS that are associated with IgG anti-GM1 or -GD1a antibodies. We hypothesised that such reversible conduction failure would be also observed in FS and related disorders.. Serial nerve conduction studies were prospectively performed in 15 patients with FS and related conditions.. Neither conduction block nor abnormal temporal dispersion was observed in any of the nerves at any point in all the patients. Conduction velocities for none of the nerves were in the demyelinating range. The amplitude of sensory nerve action potential was decreased in three FS, one FS/GBS and two BBE/GBS patients. Compound muscle action potential amplitudes were decreased in the two BBE/GBS patients. These decreases in amplitudes of sensory nerve action potential and compound muscle action potential promptly resolved without significant change in duration on serial studies.. Reversible conduction failure was seen in six of the 15 patients with FS and related disorders on serial nerve conduction studies. There were no signs of demyelination or remyelination in the 15 patients. The pathology appears to be primarily non-demyelinating. We believe these conditions form a continuous spectrum with axonal GBS.

Topics: Action Potentials; Adult; Autoantibodies; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Neural Conduction; Ophthalmoplegia

Clinical severity of Guillain-Barré syndrome (GBS) is highly variable, but the immunopathological reason is unknown.. The study was designed to show which antibody parameters are associated with disease severity in GBS patients with serum anti-GM1 IgG antibodies.. Thirty-four GBS patients with anti-GM(1) IgG antibodies were grouped into two categories according to disease severity at nadir: mild (grades 1-3 by Hughes functional scale, n=13) and severe (grades 4 and 5, n=21). Titre, affinity, fine specificity and cell binding of anti-GM(1) antibodies were obtained and compared between the two groups.. No differences in antibody titre (GM(1)-ELISA) or affinity were found between the two patient groups. In contrast, the severe group showed a significantly higher frequency (95%, vs 46% in the mild group, p=0.002) of specific (not cross-reacting with GD(1b)) anti-GM(1) antibodies. In addition, the severe group also exhibited a higher antibody binding titre to cellular GM(1).. Differences in fine specificity of antibodies are strong indications that different regions of the GM(1)-oligosaccharide are involved in antibody binding. High titres of specific anti-GM(1) antibody binding to cellular GM(1) can be explained by antigen exposure, that is, GM(1) exposes or forms mainly epitopes recognised by specific antibodies, and 'hides' those involved in binding of cross-reacting antibodies. Thus, the fine specificity of anti-GM(1) antibodies may influence disease severity by affecting antibody binding to cellular targets. Additionally, since antibody specificity studies are relatively easy to implement, fine specificity could be considered a useful predictor of disease severity.

Topics: Adolescent; Adult; Aged; Autoantibodies; Child; Child, Preschool; Chromatography, Thin Layer; Disability Evaluation; Enzyme-Linked Immunosorbent Assay; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Severity of Illness Index; Surveys and Questionnaires; Young Adult

Topics: Autoantibodies; Biomarkers; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Specimen Handling

The co-occurrence of myasthenia gravis (MG) and Guillain Barré syndrome (GBS) is uncommon with a few reported cases in the literature. There is only one reported case of MG and Miller Fisher variant of GBS. We described an 84 year old Chinese woman with underlying seropositive myasthenia gravis (MG) who presented with ophthalmoplegia, areflexia and acute neuromuscular weakness. She was proved to have co-occurrence of MG and GBS/Miller Fisher overlap syndrome with positive anti-GQ1b antibody. The unusual finding in this patient raises an interesting question on their pathogenesis with the possibility that prior activation of the immune system may predispose the development of autoantibodies against other antigens within the same set of muscles.

Topics: Aged, 80 and over; Antibodies; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Myasthenia Gravis; Serologic Tests

A 31-year-old man had optic neuritis 2 weeks after a diarrheal illness, followed by several deficits including palatal dysarthria, diplopia, ataxia, sensory dysfunction, and mild dysautonomia. Brain MRI and CSF were normal. Nerve conduction studies were initially normal and subsequently showed mild reduction in sensory amplitudes. Anti-GQ1b IgG titer was positive. Deficits resolved after treatment with IVIg. This clinical constellation represents an overlap between Miller Fisher syndrome (MFS) and the pharyngeal-cervical-brachial (PCB) variant of Guillain-Barre syndrome (GBS), along with the infrequently reported central feature of optic neuritis. Campylobacter jejuni enteritis may have triggered the syndrome by molecular mimicry. GQ1b antibodies are associated with MFS, GBS, Bickerstaff brainstem encephalitis and PCB; they form an overlapping spectrum of features, hence the anti-GQ1b syndrome.

Topics: Abducens Nerve Diseases; Adult; Diarrhea; Dysarthria; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Male; Neurologic Examination; Optic Neuritis; Palate; Speech Disorders

A 52-year-old man developed diplopia, a nasal voice, dysphagia, hoarseness and slight bilateral facial palsies. There was no ataxia, areflexia, limb weakness or sensory involvement. Serum anti-GQ1b IgG antibody was present. Treatment with intravenous immunoglobulin started, and the patient responded with a rapid resolution of symptoms. The diagnosis is consistent with polyneuritis cranialis which is considered to be a Guillain-Barre syndrome variant, a forme fruste, but very rare. The diagnosis can be difficult and a thorough investigation is required. Electrophysiological examination, laboratory evaluations, imaging and cerebrospinal fluid examination are often required in the investigations. Cranial neuropathy can be the presentation of many disorders. Determination of anti-ganglioside antibodies as anti-GQ1b is valuable to the diagnosis, and shows the association with the Guillain-Barre syndrome.

Topics: Autoantibodies; Cranial Nerve Diseases; Diagnosis, Differential; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Neuritis

To determine the epitopes of ganglioside complexes (GSCs) containing GQ1b or GT1a, we investigated their reactivity to GSCs consisting of asialo-GM1 (GA1) and GQ1b or GT1a using IgG anti-GQ1b- or anti-GT1a-positive sera. Nine anti-GQ1b-positive sera had higher activity to GA1/GQ1b than to GQ1b, only five of which reacted with GM1/GQ1b and GD1b/GQ1b. Five of 14 sera positive for GA1/GT1a and GM1/GT1a were negative for GA1/GQ1b and GM1/GQ1b. Sialic acids attached to the internal galactose of gangliotetraose can influence the reactivity of anti-GSC antibodies. Screening for antibodies to GSCs containing GA1 is useful for elucidation of the antibody-mediated pathophysiology.

Topics: Antibodies, Monoclonal; Autoantibodies; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome

We described an overlap syndrome associating Miller Fisher syndrome (MFS) and acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Furthermore, the patient presented unusual neurological manifestations including headache, T10 sensory level, urinary urgency, and gadolinium enhancement of the spinal roots. One year follow-up was characterized by clinical recovery and persistent high rates of anti-GQ1b, -GD1b and -GT1b antibodies. Our case suggests broad phenotype of persistent antigangliosides antibodies.

Topics: Adult; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins; Treatment Outcome

A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. Neurological examination showed ophthalmoplegia, facial paralysis, tetraplegia and loss of deep tendon reflexes. Babinski reflex was positive on the left and there were bilateral flexor withdrawal reflexes. He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. Anti-GQ1b IgG antibody was selectively elevated in serum, and CSF protein was elevated to 53 mg/dl with cell count of 12/mm3. Nerve conduction study showed decreased amplitude of compound motor action potentials in all extremities, and no response in facial muscles. Cranial MRI showed no abnormalities whereas EEG was severely abnormal with lack of posterior dominant rhythm and the presence of continuous diffuse theta-waves. This case presented clinical characteristics of three syndromes concurrently-Fisher syndrome, Bickerstaff brainstem encephalitis, and Guillain-Barré syndrome-that may be collectively called 'anti-GQ1b IgG antibody syndrome'. The unique feature of the present case was development of deep coma and ballism-like movements, associated with selective increase of serum anti-GQ1b IgG antibody. It is thus conceivable that anti-GQ1b IgG antibody might underlie the pathogenesis of all three conditions.

Topics: Adult; Brain Stem; Dyskinesias; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Male; Miller Fisher Syndrome

Topics: Autoantibodies; Gangliosides; Guillain-Barre Syndrome; Humans; Immunologic Factors; Neuromuscular Junction; Peripheral Nerves

Serum antibodies to GQ1b are associated with Miller Fisher syndrome (MFS) and Guillain-Barré syndrome (GBS) with ophthalmoplegia. Antibodies to ganglioside complexes (GSCs) have not yet been examined in a large population of patients with MFS or GBS. This study aimed to determine the clinical significance of antibodies to GSCs in MFS and GBS.. The study investigated serum anti-GSC antibodies and the clinical features in 64 MFS patients, 53 GBS patients with ophthalmoplegia (GBS-OP(+)) and 53 GBS patients without ophthalmoplegia (GBS-OP(-)).. Thirty patients with MFS (47%), 25 with GBS-OP(+) (47%) and none with GBS-OP(-) had antibodies to GSCs containing GQ1b or GT1a. Patients with MFS and GBS-OP(+) were subdivided according to the antibody reactivities; patients with antibodies specific to GQ1b and/or GT1a (without anti-GSCs antibodies) were placed in Group 1, those with antibodies against GSCs with a total of two sialic acids in the terminal residues, such as GQ1b/GM1, were placed in Group 2, and those with antibodies against GSCs with a total of three sialic acids in the terminal residue, such as GQ1b/GD1a, were placed in Group 3. In MFS, sensory disturbances were infrequent in Group 2 compared with the other groups (p<0.0001). Antibodies specific to GQ1b were observed more often in MFS than in GBS-OP(+) (p = 0.0002).. IgG antibodies to GSCs containing GQ1b or GT1a were closely associated with the development of ophthalmoplegia in GBS, as well as MFS. Both GQ1b and clustered epitopes of GSCs containing GQ1b or GT1a may be prime target antigens for MFS and GBS-OP(+).

Topics: Adult; Antibodies; Antibody Specificity; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

The capacity of ganglioside-specific autoantibodies to recruit leukocyte effector functions was studied. Serum samples from 87 patients with Guillain-Barré (GBS) or Miller Fisher syndrome (MFS), containing GM1-, GQ1b-, or GD1b-specific IgG or IgA, were tested for leukocyte activating capacity. Ganglioside-specific IgG antibodies generally induced leukocyte activation, irrespective of specificity. The magnitude of leukocyte degranulation correlated with GM1- and GQ1b-specific IgG titers, but not with disease severity. Finally, GM1-specific IgA activated leukocytes through the IgA receptor, FcalphaRI (CD89). Therefore, both ganglioside-specific IgG and IgA can recruit leukocyte effector functions, which may be relevant in the pathogenesis of GBS and MFS.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antibody Specificity; Antigens, CD; Autoantibodies; Cell Degranulation; Child; Child, Preschool; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin A; Immunoglobulin G; Leukocytes; Male; Middle Aged; Miller Fisher Syndrome; Receptors, Fc

Topics: Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Electrodiagnosis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Middle Aged; Neural Conduction; Paresthesia; Peripheral Nerves; Predictive Value of Tests; Treatment Outcome

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Intracellular Signaling Peptides and Proteins; Mental Status Schedule; Neurologic Examination; Neuropeptides; Ophthalmoplegia; Orexins

The Miller Fisher syndrome, Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis are related conditions in which anti-GQ1b antibody positivity occur in varied frequencies. This report demonstrates the presence of corticobulbar and corticospinal dysfunction in BBE, by means of a novel transcranial magnetic stimulation technique. It further supports the presence of protean manifestations in anti-GQ1b IgG antibody-positive spectrum of disorders.

Topics: Adult; Autoantibodies; Brain Stem; Dysarthria; Encephalitis; Evoked Potentials, Motor; Gangliosides; Guillain-Barre Syndrome; Humans; Hypoglossal Nerve; Hypoglossal Nerve Diseases; Male; Motor Neurons; Neural Conduction; Neurologic Examination; Pyramidal Tracts; Reaction Time; Reflex, Abnormal; Remission, Spontaneous; Syndrome; Tongue; Transcranial Magnetic Stimulation

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain-Barré syndrome or mild form of Fisher syndrome.

Topics: Abducens Nerve; Abducens Nerve Diseases; Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Child; Child, Preschool; Diagnosis, Differential; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Infant; Infections; Male; Middle Aged; Miller Fisher Syndrome; Plasmapheresis; Predictive Value of Tests; Reflex, Abnormal; Steroids

We developed testing kits for anti-GM1 and anti-GQ1b IgG antibodies and examined their utilities in supporting the diagnosis of Guillain-Barré syndrome (GBS) and Fisher syndrome (FS). Anti-GM1 antibody was detected in 49% of 95 patients with GBS and in 5% or less of disease and normal controls. Anti-GQ1b antibody was detected in 85% of 55 patients with FS, whereas in none of the controls. Eight GBS patients, in whom anti-GM1 IgG antibody was judged negative using the kit, were found to have other anti-ganglioside IgG antibodies. Four of them showed ophthalmoplegia and had anti-GQ1b IgG antibody. Detection of anti-GM1 IgG antibody in GBS and of anti-GQ1b IgG antibody in FS within one week after the disease onset were significantly more frequent compared to albuminocytologic dissociation in the cerebrospinal fluids (GBS, 58% vs 32%; FS, 89% vs 20%). These findings indicate that our testing kits are useful for supporting the early diagnosis of GBS and FS.

Topics: Adult; Autoantibodies; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Reagent Kits, Diagnostic

Serum antibody activities to mixtures of a ganglioside and various phospholipids were compared with those to a ganglioside alone in 30 anti-GM1 IgG-positive GBS patients and 30 anti-GQ1b IgG-positive Miller Fisher syndrome (MFS) patients. Anti-GM1-positive sera had higher antibody reactivities against a mixture of GM1 and several phospholipids including PA, PI and PS, than against GM1 alone. In contrast, in case of anti-GQ1b antibody, no phospholipid provided significant enhancement. Sphingomyelin provided decrease of the activity for both anti-GM1 and anti-GQ1b IgG. The effects of phospholipids must be considered to determine the pathogenetic role of antiganglioside antibodies in GBS and MFS.

Topics: Adjuvants, Immunologic; Binding Sites, Antibody; Cardiolipins; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Lysophosphatidylcholines; Lysophospholipids; Phosphatidic Acids; Phosphatidylcholines; Phosphatidylinositols; Phosphatidylserines; Phospholipids; Sphingomyelins

Ganglioside epitopes on Campylobacter jejuni are hypothesized as the key to the development and characterization of Guillain-Barré syndrome (GBS), but a comprehensive theory has yet to be established. A C jejuni gene, cst-II, involved in the biosynthesis of ganglioside-like lipo-oligosaccharide, shows a polymorphism (Asn/Thr51) that affects ganglioside epitopes.. To examine the hypothesis that this polymorphism determines autoantibody reactivity, and thereby neurologic presentations in GBS.. C jejuni isolates were collected from 105 GBS (including its variants) and 65 uncomplicated enteritis patients. The authors examined the frequency of cst-II and polymorphism (Asn/Thr51) in connection with the bacterial ganglioside epitopes, autoantibody reactivities against GM1, GD1a, and GQ1b, and patients' neurologic findings.. Neuropathic strains more frequently had cst-II, in particular cst-II (Thr51), than did enteritic ones (85% vs 52%; p < 0.001). Strains with cst-II (Asn51) regularly expressed the GQ1b epitope (83%), whereas those with cst-II (Thr51) had the GM1 (92%) and GD1a (91%) epitopes. The presence of these bacterial epitopes in neuropathy patients corresponded to autoantibody reactivity. Patients infected with C jejuni (Asn51) more often were positive for anti-GQ1b IgG (56% vs 8%; p < 0.001) and had ophthalmoparesis (64% vs 13%; p < 0.001) and ataxia (42% vs 11%; p = 0.001). Patients who had C jejuni (Thr51) more frequently were positive for anti-GM1 (88% vs 35%; p < 0.001) and anti-GD1a IgG (52% vs 24%; p = 0.006) and had limb weakness (98% vs 71%; p < 0.001).. The genetic polymorphism of C jejuni determines autoantibody reactivity as well as the clinical presentation of Guillain-Barré syndrome (GBS), possibly through modification of the host-mimicking molecule. The GBS paradigm is the first to explain the detailed pathogenesis of a postinfectious, autoimmune-mediated, molecular mimicry-triggering disorder.

Topics: Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Epitopes; G(M1) Ganglioside; Gangliosides; Gene Expression Regulation, Bacterial; Guillain-Barre Syndrome; Humans; Lipopolysaccharides; Molecular Mimicry; Mutation; Polymorphism, Genetic; Species Specificity

Acute and chronic autoimmune neuropathies, including Guillain-Barré syndromes (GBS) are often characterized by the presence of autoantibodies that react with neural gangliosides. Evidence from human and animal studies indicates that anti-ganglioside antibodies play a primary neuropathogenic role, and their rapid elimination from the circulation through specific immunoadsorption therapy thus has the potential to ameliorate the course of the disease. Here we have tested this therapeutic principle in the Miller Fisher variant of GBS that is associated serologically with acute phase anti-GQ1b ganglioside immunoglobulin G (IgG) antibodies, and in chronic ataxic neuropathies associated with persistently elevated immunoglobulin M (IgM) antibodies that react with GQ1b, GD3 and other disialylated gangliosides. Human and mouse anti-GQ1b IgG and IgM antibodies may also react with GD3, suggesting the shared terminal disialoside epitope could be involved in antibody binding. We thus synthesized the terminal trisaccharide, NeuAc(alpha2-8)NeuAc(alpha2-3)Gal common to GQ1b and GD3, and conjugated it to bovine serum albumin (BSA). This disialylgalactose glycoconjugate (DSG-BSA) binds anti-GQ1b antibodies in 32/58 (55%) human sera containing IgG or IgM anti-GQ1b antibodies at titres up to 1/130 000; it also binds a wide range of mouse monoclonal anti-GQ1b and -GD3 antibodies. When conjugated to Sepharose as mock therapeutic immmunoaffinity columns, the immobilized trisaccharide (DSG-Sepharose) eliminates anti-GQ1b antibodies from positive sera in proportion to their level of binding to DSG-BSA. Oligosaccharide-specific immunoadsorption therapy thus provides a new therapeutic approach to anti-GQ1b antibody-associated syndromes that could be applied to clinical practice. Furthermore, modification of the immobilized oligosaccharide epitopes to incorporate other glycan structures may allow this approach to be adapted to other forms of autoimmune neuropathy associated with uniform anti-glycolipid antibody profiles.

Topics: Animals; Antibodies, Monoclonal; Antigen-Antibody Reactions; Gangliosides; Glycolipids; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Immunosorbent Techniques; Immunosorbents; Mice; Mice, Inbred Strains; Mice, Knockout

Fisher syndrome (FS), a variant of Guillain-Barré syndrome (GBS), is a rare disorder, and there are few reported studies of a large number of patients with FS. Cerebrospinal fluid (CSF) albuminocytological dissociation was found in 59% of 123 FS patients during the first 3 weeks of illness, while serum anti-GQ1b IgG antibody was positive in 85%. Whereas the incidence of CSF albuminocytological dissociation increased from the first to second weeks in FS, anti-GQ1b IgG antibody peaked in the first week, but there was no CSF albuminocytological dissociation. Statistically, anti-GQ1b antibody testing was superior to a CSF examination in supporting a diagnosis of FS during the first 3 weeks of illness, especially in the first week.

Topics: Adolescent; Adult; Age Factors; Aged; Aged, 80 and over; Albumins; Analysis of Variance; Antibodies, Anti-Idiotypic; Child; Child, Preschool; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Infant; Infant, Newborn; Male; Middle Aged; Miller Fisher Syndrome; Statistics, Nonparametric; Time Factors

Compared with 87 unventilated patients with Guillain-Barré syndrome (GBS), 44 ventilated patients with GBS more frequently had multiple cranial nerve involvement (91 vs 50%; p < 0.001) and IgG anti-GQ1b antibody (27 vs 8%; p = 0.006). In GBS patients without ophthalmoparesis, the presence of IgG anti-GQ1b antibody was associated with respiratory failure (12 [3/25] vs 0% [0/67]; p = 0.04). The presence of the antibody may be a factor predictive of respiratory failure in GBS.

Topics: Adult; Antibodies, Anti-Idiotypic; Disease Progression; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Respiration, Artificial; Respiratory Insufficiency

Guillain-Barré syndrome is a postinfectious, autoimmune neuropathy resulting in neuromuscular paralysis. Auto-antibodies, often induced by bacterial infection, bind to human gangliosides possessing monosialoside and diasialoside epitopes and impair the function of nerve junctions, where these ganglioside structures are highly enriched. Truncated gangliosides representive of GD3, GQ1b and GM2 epitopes have been synthesized as methyl glycosides and as a glycosides of an eleven carbon tether. The synthetic oligosaccharide ligands are structural mimics of these highly complex ganglioside epitopes and via their ability to neutralize or remove auto-antibodies have the potential for therapy, either as soluble blocking ligands administered systemically, or as immuno-affinity ligands for use as extracorporeal immunoadsorbents.

Topics: Antibodies, Monoclonal; Antigen-Antibody Reactions; Carbohydrate Sequence; G(M2) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunosorbent Techniques; Immunosorbents; Ligands; Molecular Sequence Data; Neuromuscular Nondepolarizing Agents; Oligosaccharides

Topics: Antibody Specificity; Autoantibodies; Autoantigens; Bulbar Palsy, Progressive; Cross Reactions; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Logistic Models; Ophthalmoplegia; Predictive Value of Tests; Respiration, Artificial; Respiratory Insufficiency

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

A 37-year-old man developed an acute encephalitic condition after respiratory infection. His condition rapidly deteriorated, and he experienced ophthalmoplegia, tetraplegia, loss of brainstem reflexes and deep tendon reflexes, and deep coma. Electrophysiological evaluations indicated involvement of the peripheral nerve as well as the brainstem. Follow-up studies found acute progression of peripheral nerve damage. Serum anti-GQ1b IgG antibody was present. The initial condition was diagnosed as Bickerstaff's brainstem encephalitis, and subsequent overlapping of Guillain-Barré syndrome probably was responsible for the clinical deterioration. When unusual worsening is observed in clinically suspected encephalitis, neurologists must take into account the possibility of associated Guillain-Barré syndrome and related disorders.

Topics: Adult; Antibodies; Blotting, Western; Brain Diseases; Electrophysiology; Encephalitis; Evoked Potentials, Auditory; Follow-Up Studies; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Neural Conduction; Ophthalmoplegia; Quadriplegia

Visceral leishmaniasis is an endemic parasitic infection rarely observed in association with Guillain-Barré syndrome in immunocompetent patients. A 40-year-old immunocompetent woman was admitted to our unit with recent onset difficulty in walking. The neurological examination and electrophysiological study led to the diagnosis of Guillain-Barré syndrome. During hospitalization, she developed cytopenia involving all three lines revealing visceral leishmaniasis. A few cases of visceral leishmaniasis with neuropathy have been reported, mainly in tropical regions. Neuropathological manifestations of visceral leishmaniasis are probably underestimated. The question is whether Guillain-Barré syndrome and visceral leishmaniasis are causally related.

Topics: Adult; Antibodies; Diagnosis, Differential; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunocompetence; Leishmaniasis, Visceral; Lymphocytes; Magnetic Resonance Imaging; Neural Conduction; Peripheral Nerves

We report a 30-year-old woman who presented symptoms of oropharyngeal palsy and glove-stocking type sensory disturbance followed by acute cerebellar ataxia, the syndrome of inappropriate secretion of antidiuretic hormone (SIADH), gastroenteric symptoms, urinary disturbance and orthostatic hypotension. She did not have any preceding infection. She was diagnosed as having Guillain-Barré syndrome with autonomic failure. Autonomic failure such as sinus tachycardia and nocturnal ventricular arrhythmia in addition to motor and sensory dysfunction was palliated by immunoadsorption. During the course of her illness, there were elevations of antiganglioside antibodies to GT1a and GQ1b in the IgG subclass, and to GD1b and GQ1b in the IgM subclass. The elevation of anti-GD1b antibody and anti-GQ1b antibody may be pathologically related to autonomic failure, cerebellar ataxia and SIADH.

Topics: Adult; Autoantibodies; Autonomic Nervous System Diseases; Cerebellar Ataxia; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Inappropriate ADH Syndrome

Topics: Diagnosis, Differential; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Middle Aged; Ophthalmoplegia

Topics: Autoantibodies; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Lambert-Eaton Myasthenic Syndrome; Middle Aged; Neuromuscular Junction; Presynaptic Terminals

Topics: Adolescent; Adult; Aged; Autoantibodies; Autoimmune Diseases of the Nervous System; Brain Stem; Child; Child, Preschool; Diagnosis, Differential; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

Sera from 40 patients with Guillain-Barré syndrome (GBS), including the subtypes acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor and sensory axonal neuropathy (AMSAN), and Miller Fisher syndrome (MFS) were examined for the presence of anti-ganglioside antibodies using the ganglioside agglutination assay, and the enzyme-linked immunosorbent assay (ELISA). In the ELISA system, sera were tested for IgM and IgG antibodies to GM1, GM2, GD1a, GD1b, GT1b, and GQ1b gangliosides. Antibodies to gangliosides were detected in 21 (53%) of the GBS patients by agglutination assay and in 17 (43%) of the patients by ELISA. Some of the sera reacted with more than one ganglioside. Antibodies were not found in the control sera that were studied. The agglutination assay may be useful for rapid screening of GBS sera for antibodies to multiple gangliosides.

Topics: Agglutination Tests; Antibodies; Axons; Enzyme-Linked Immunosorbent Assay; G(M1) Ganglioside; G(M2) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulin M; Peripheral Nerves; Reproducibility of Results

To clarify the nosological relation among Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brain stem encephalitis (BBE), and acute ophthalmoparesis without ataxia. Serum samples from patients with each condition often have anti-GQ1b IgG antibody.. Information on antecedent illness, initial symptoms, neurological signs during the illness, and CSF findings were reviewed in 194 patients with anti-GQ1b IgG. It was determined whether overlapping MFS and GBS (MFS/GBS), as well as overlapping BBE and GBS (BBE/GBS), is explained by the combined action of anti-GQ1b IgG and anti-GM1 or anti-GD1a IgG, serological markers of GBS.. Based on the diagnostic criteria, all the patients with acute ophthalmoparesis, MFS, MFS/GBS, BBE/GBS, and BBE had external ophthalmoplegia; all the patients with MFS, MFS/GBS, or GBS had hyporeflexia or areflexia; and all those with MFS and BBE showed ataxia. Tendon reflexes were decreased or absent in 91% of those with BBE/GBS, 67% of those with BBE, and 53% of those with acute ophthalmoparesis. Ataxia was present in 68% of the patients with MFS/GBS and 45% of those with BBE/GBS. Antecedent illness caused by upper respiratory tract infection had occurred in 60% to 80% of these patients, and CSF albuminocytological dissociation in 25% to 75%. Anti-GM1 or anti-GD1a IgG was present in 50% of those with GBS, 35% of those with MFS/GBS, 27% of those with BBE/GBS, 16% of those with MFS, and 8% of those with BBE.. These findings together with the common autoantibody (anti-GQ1b IgG) suggest that a common autoimmune mechanism functions in the pathogenesis of these illnesses. In a larger study, it was confirmed clinically that MFS, GBS, BBE, and acute ophthalmoparesis are closely related, forming a continuous range. This is supported by the immunological findings. The term "anti-GQ1b IgG antibody syndrome" is not intended to be used as a clinical diagnosis, but recognition of this syndrome is useful for understanding the aetiological relation among the various illnesses and for introducing the established treatments of GBS for use with other conditions.

Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

Campylobacter jejuni infections are thought to induce antiganglioside antibodies in patients with Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) by molecular mimicry between C. jejuni lipopolysaccharides (LPS) and gangliosides. We used purified LPS fractions from five Campylobacter strains to induce antiganglioside responses in rabbits. The animals that received injections with LPS from GBS-associated strains developed anti-GM1 and anti-GA1 antibodies. Animals injected with LPS from one MFS-related C. jejuni strain produced anti-GQ1b antibodies. Rabbits that were injected with Penner O:3 LPS had a strong anti-LPS response, but no antiganglioside reactivity was observed. The antiganglioside specificity in the rabbits reflected the specificity in the patients from whom the strains were isolated. In conclusion, our results indicate that an immune response against GBS- and MFS-associated C. jejuni LPS results in antiganglioside antibodies. These results provide strong support for molecular mimicry as a mechanism in the induction of antiganglioside antibodies following infections.

Topics: Adult; Animals; Antibodies, Bacterial; Campylobacter jejuni; Child; Epitopes; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunization; Lipopolysaccharides; Male; Middle Aged; Miller Fisher Syndrome; Rabbits

Topics: Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Ophthalmoplegia

Topics: Autoantibodies; Diagnosis, Differential; Encephalitis, Viral; Gangliosides; Guillain-Barre Syndrome; Humans; Mesencephalon; Miller Fisher Syndrome; Ophthalmoplegia; Rhombencephalon

Elevated anti-ganglioside antibody levels mainly of anti-GM1 and anti-GD1a specificities have been reported in THE serum of patients with Guillain-Barré syndrome (GBS). The relevance of anti-ganglioside antibodies other than anti-GM1 and anti-GD1a IgG antibodies and the temporal profile of anti-ganglioside antibodies in GBS is less clear. We studied serum antibodies to GM1, GD1a, GD1b, GQ1b, sulfatide and cardiolipin of the IgM, IgG and IgA classes over the course of GBS in patients who were untreated or treated with high dose intravenous immunoglobulin (IvIg). Antibodies to GD1b, GQ1b, sulfatide and cardiolipin were not detected in the sera of the GBS patients examined in this study. Anti-GM1 IgG titers peaked around 40 days and anti-GD1a IgM around 90 days after GBS onset. Titers of anti-GM1 IgG antibodies decreased following IvIg treatment. Patients with antibody peaks, defined as fivefold or higher increase in antibody titer compared to the lowest antibody titer over the course of GBS, had higher disability scores during the first two weeks of GBS and a worse clinical outcome (anti-GM1 IgG and anti-GD1a IgM antibody peaks) and axonal damage (anti-GD1a IgM antibody peaks), compared to patients without peak antibody titers. Anti-GM1 IgG and anti-GD1a IgM antibodies are thus strongly associated with more severe- and predominantly axonal cases of GBS. The appearance of anti-GM1 IgG and anti-GD1a antibody peaks in the serum after the termination of the acute phase of GBS suggests that these antibodies are produced secondary to nerve damage in GBS. The data does not exclude the possibility that secondarily secreted anti-GM1 IgG and anti-GD1a IgM antibodies may themselves be biologically active and play a role in disease propagation and/or recovery from disease in some patients with GBS.

Topics: Antibodies; Axons; Cardiolipins; Disease Progression; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin A; Immunoglobulin G; Immunoglobulin M; Immunoglobulins, Intravenous; Male; Middle Aged; Sulfoglycosphingolipids; Time Factors

We described a 70-year-old woman with overlapping Fisher's syndrome (FS) and Guillain-Barré syndrome (GBS), from whom Campylobacter jejuni had been isolated. In typical FS as well as GBS with ophthalmoplegia and acute ophthalmoparesis without ataxia, serum anti-GQ1b IgG antibody often is detected and ophthalmoplegia is characterized by the predominant abducens palsy. This patient, however, showed marked oculomotor nerve disturbance. Serum anti-GQ1b IgG antibody was negative and IgG antibodies against GM1, GM1b, and GD1a were strongly positive. Although FS and overlap of FS/GBS have been reported to be associated with PEN2 of C. jejuni, the isolate from our case belonged to PEN 19. C. jejuni serotype may be associated with clinical manifestations and anti-ganglioside antibody species.

Topics: Aged; Campylobacter Infections; Campylobacter jejuni; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Miller Fisher Syndrome

The authors reviewed the medical records of seven patients with anti-GQ1b immunoglobulin G (IgG) who had no or minimal external ophthalmoplegia but showed ataxia. The clinical features of the patients were consistent with the ataxic form of Guillain-Barré syndrome (GBS). Anti-GQ1b IgG antibodies from the patients, as well as from those with Miller Fisher syndrome (MFS), were absorbed by GT1a. The finding that ataxic GBS and MFS have in common an autoantibody with the same fine specificity suggests that they form a continuous spectrum.

Topics: Adult; Antibody Specificity; Ataxia; Autoantibodies; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Neurologic Examination; Neuropsychological Tests

Topics: Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome

Serum IgG antibody against LM1, the predominant ganglioside in the human peripheral nerve myelin, was found in 7 out of 140 patients with Guillain-Barré syndrome (GBS) in the acute phase, 1 out of 33 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), and 2 out of 47 patients with Miller Fisher syndrome (MFS). Anti-LM1 IgM antibody was detected only in 2 patients, each with GBS and MFS. The clinical and electrophysiological features of the seven GBS patients with anti-LM1 IgG antibody in the serum were investigated. Six patients recovered to grade 1 within one month of the onset of neuropathy. Electrophysiological studies revealed demyelination in five patients, of which one had axonal damage in addition, whereas sufficient evidence of demyelination or axonal degeneration was not observed in the remaining two. Five had a respiratory tract infection before the onset of neuropathy, and also had serum anti-GQ1b IgG antibody. IgG antibody against LM1 might be involved in the pathogenetic mechanisms of GBS, as a possible demyelinating factor. Presence of both anti-GQ1b and anti-LM1 antibodies may be associated with some infectious agent(s) affecting the respiratory tract.

Topics: Antibodies, Bacterial; Autoantibodies; Campylobacter jejuni; Chromatography, Thin Layer; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Miller Fisher Syndrome; Myelin Sheath; Peripheral Nerves; Peripheral Nervous System Diseases; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating; Respiratory Tract Infections

Topics: Aged; Autoantibodies; Campylobacter Infections; Campylobacter jejuni; Cranial Nerve Diseases; Diagnosis, Differential; Diarrhea; Feces; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Paralysis