Anti-glycolipid antibodies in patients with neuropathy: A diagnostic assessment

Johannis, Wibke; Renno, Joerg H.; Klatt, Andreas R.; Wielckens, Klaus

doi:10.1016/j.jocn.2013.07.041

Cited by 6 publications

(4 citation statements)

References 25 publications

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“…The incidence of AGAs in healthy individuals in control groups appear to range from 1 to 9%. 75,76 Sometimes, IgM class anti-GM1 antibodies can be found in the sera of healthy individuals after bacterial infections, while the presence of IgG antibodies, especially of IgG1 and IgG3 subclasses, are generally linked to pathological processes. 77…”

Section: Immune-mediated Diseasesmentioning

confidence: 99%

Physiology of gangliosides and the role of antiganglioside antibodies in human diseases

2020

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Gangliosides are structurally and functionally polymorphic sialic acid containing glycosphingolipids that are widely distributed in the human body. They play important roles in protecting us against immune attacks, yet they can become targets for autoimmunity and act as receptors for microbes, like the influenza viruses, and toxins, such as the cholera toxin. The expression patterns of gangliosides vary in different tissues, during different life periods, as well as in different animals. Antibodies against gangliosides (AGA) can target immune attack e.g., against neuronal cells and neutralize their complement inhibitory activity. AGAs are important especially in acquired demyelinating immune-mediated neuropathies, like Guillain-Barré syndrome (GBS) and its variant, the Miller-Fisher syndrome (MFS). They can emerge in response to different microbial agents and immunological insults. Thereby, they can be involved in a variety of diseases. In addition, antibodies against GM3 were found in the sera of patients vaccinated with Pandemrix®, who developed secondary narcolepsy, strongly supporting the autoimmune etiology of the disease.

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Section: Immune-mediated Diseasesmentioning

confidence: 99%

Physiology of gangliosides and the role of antiganglioside antibodies in human diseases

2020

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“…Another limitation is that data were not available detailing how many patients with no polyneuropathy presented with disialosyl antibodies in our 14 recruiting centers. Nonetheless, estimates may be inferred from the data of healthy control groups from several studies, and the incidence of anti‐ganglioside antibodies in healthy individuals ranges from 1% to 9% [23–25].…”

Section: Discussionmentioning

confidence: 99%

“…Secondly, the multicentric design led to absent standardized data collection and clinical evaluation. For instance, clinical follow-up was highly variable between each recruiting center, with centers indiscriminately using the Rasch-built Overall Disability Scale, the to 9% [23][24][25].…”

Section: Limitations Of Our Studymentioning

confidence: 99%

Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

Peillet

Adams

Attarian

et al. 2022

Euro J of Neurology

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Background and purpose In this retrospective study involving 14 university hospitals from France and Switzerland, the aim was to define the clinicopathological features of chronic neuropathies with anti‐disialosyl ganglioside immunoglobulin M (IgM) antibodies (CNDA). Results Fifty‐five patients with a polyneuropathy evolving for more than 2 months and with at least one anti‐disialosyl ganglioside IgM antibody, that is, anti‐GD1b, ‐GT1b, ‐GQ1b, ‐GT1a, ‐GD2 and ‐GD3, were identified. Seventy‐eight percent of patients were male, mean age at disease onset was 55 years (30–76) and disease onset was progressive (82%) or acute (18%). Patients presented with limb sensory symptoms (94% of cases), sensory ataxia (85%), oculomotor weakness (36%), limb motor symptoms (31%) and bulbar muscle weakness (18%). Sixty‐five percent of patients had a demyelinating polyradiculoneuropathy electrodiagnostic profile and 24% a sensory neuronopathy profile. Anti‐GD1b antibodies were found in 78% of cases, whilst other anti‐disialosyl antibodies were each observed in less than 51% of patients. Other features included nerve biopsy demyelination (100% of cases), increased cerebrospinal fluid protein content (75%), IgM paraprotein (50%) and malignant hemopathy (8%). Eighty‐six percent of CNDA patients were intravenous immunoglobulins‐responsive, and rituximab was successfully used as second‐line treatment in 50% of cases. Fifteen percent of patients had mild symptoms and were not treated. CNDA course was progressive (55%) or relapsing (45%), and 93% of patients still walked after a mean disease duration of 11 years. Conclusion Chronic neuropathies with anti‐disialosyl ganglioside IgM antibodies have a recognizable phenotype, are mostly intravenous immunoglobulins‐responsive and present with a good outcome in a majority of cases.

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“…Positive GQ1b antibodies can cause ophthalmoplegia and ataxia and can also bind to GQ1b expressed in the brainstem, resulting in consciousness disorder accompanied by a pyramid sign. [ 16 , 17 ] Studies have shown a high positive rate of IgG antibodies to GQ1b in patients with MFS (83–95%) and BBE (66–68%). [ 18 ] In 2013, Shahrizaila [ 19 ] et al proposed the concept of antiGQ1b antibody syndrome, with the deepening of the understanding of GQ1b antibody, and it is increasingly recognized that MFS and BBE are 2 ends of the antiGQ1b antibody syndrome continuum, and can manifest as various combinations of central and peripheral nervous system involvement.…”

Section: Discussionmentioning

confidence: 99%

AntiGQ1b antibody positive with MFS/GBS overlapped syndrome with diplopia and hemiplegia onset: Case report and retrospective analysis

Zhao

et al. 2022

Medicine

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Rationale: GBS and MFS have been divided into several subtypes, constituting a series of independent and overlapping syndromes that share similar pathophysiology, leading to common clinical features, including history of previous infection, single-phase course, symmetry, skull or limbs weakness, CFS albumin cell separation (high protein, normal cell count), antiganglioside antibodies and axon, or evidence of demyelinating neuropathy neurophysiology. Part of the MFS in patients with clinical manifestations may be complicated, and even symptoms are not typical. A few patients may overlap with BBE or GBS. Patient concerns: Most patients with MFS/GBS overlap syndrome have a good prognosis, and a few patients may experience fluctuations or re-exacerbations. In most patients, after treatment, their neurological function basically recovers within a few weeks or months. Diagnosis interventions: The patient had ophthalmoplegia, ataxia, weak force, and protein-cell separation in cerebrospinal fluid during the development of the disease. The diagnosis of MFS overlapped with typical GBS was considered. The CSF specific IgG oligoclonal zone and anti-Sulfatide antibody were positive. Anti-GT1a IgG was positive. Anti-GQ1b IgG was positive, which supported the diagnosis of GBS spectrum disorders. According to their common immunological basis, plasma exchange or intravenous immunoglobulin (IVIG) therapy is recommended, which can effectively improve the symptoms and shorten the course of the disease. Outcomes: After treatment with glucocorticoids and gamma globulin, the symptoms improved and the patient was discharged. Lessons: MFS/GBS Superimposed syndrome is a rare clinical disease. Therefore, more attention should be paid to early diagnosis and treatment of similar patients to avoid misdiagnosis. Cerebral spinal fluid (CFS) examination, neuroelectrophysiology, and GQ1b antibody detection can be used to confirm the diagnosis.

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Anti-glycolipid antibodies in patients with neuropathy: A diagnostic assessment

Cited by 6 publications

References 25 publications

Physiology of gangliosides and the role of antiganglioside antibodies in human diseases

Physiology of gangliosides and the role of antiganglioside antibodies in human diseases

Anti‐disialosyl‐immunoglobulin M chronic autoimmune neuropathies: a nationwide multicenter retrospective study

AntiGQ1b antibody positive with MFS/GBS overlapped syndrome with diplopia and hemiplegia onset: Case report and retrospective analysis

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