Ocular Flutter, Generalized Myoclonus, and Trunk Ataxia Associated With Anti-GQ1b Antibodies

Zaro-Weber, Olivier; Galldiks, Norbert; Dohmen, Christian; Fink, Gereon R.; Nowak, Dennis A.

doi:10.1001/archneur.65.5.659

Cited by 36 publications

(16 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…A detailed peripheral nerve assessment done for the 19-year-old woman with SPS proved unremarkable, thus suggesting that detected ganglioside antibodies did not have a pathogenic action on peripheral nerves. However, as reported previously, ganglioside antibodies may be associated with pure CNS findings without profoundly affecting peripheral nerve functions [25,26]. All ganglioside antibody positive patients in our cohort had relatively high maximum mRS.…”

Section: Discussionsupporting

confidence: 80%

Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis

Gürsoy¹,

Selçuk²,

Tunc³

et al. 2021

Turk J Med Sci

View full text Add to dashboard Cite

Background/aim: Ganglioside antibodies are identified not only in patients with inflammatory neuropathies but also several central nervous system disorders and paraneoplastic neuropathies. Our aim was to investigate whether ganglioside antibodies are found in autoimmune encephalitis patients and may function as a diagnostic and prognostic biomarker.Materials and methods: Sera and cerebrospinal fluid (CSF) samples of 33 patients fulfilling the criteria for probable autoimmune encephalitis were collected within the first week of clinical manifestation. None of the patients had evident symptoms and findings of peripheral polyneuropathy. Well-characterized anti-neuronal and paraneoplastic antibodies were investigated in sera and CSF and anti-ganglioside (anti-GM1, GM2, GM3, GD1a, GD1b, GT1b and GQ1b) IgG and IgM antibodies were measured in sera using commercial immunoblots.Results: Twenty-eight of 33 autoimmune encephalitis patients displayed antibodies against neuronal surface or onco-neural antigens with N-methyl-D-aspartate receptor (NMDAR), glutamic acid decarboxylase (GAD) and Hu antibodies being the most prevalent. While no anti-ganglioside IgG antibodies were found, 4 patients (2 anti-NMDAR+, 1 anti-GAD+ and 1 antibody negative) with autoimmune limbic encephalitis displayed anti-GM1, anti-GM2, anti-GM3 or anti-GQ1b IgM antibodies. There was no apparent association between anti-ganglioside positivity and clinical and demographic features. Conclusion:Serum ganglioside IgM antibodies may infrequently emerge during the clinical course of autoimmune limbic encephalitis without evident polyneuropathy.Absence of the IgG response suggests that these antibodies might have developed as a 2 hyperacute immune response to neuro-axonal destruction. Nevertheless, potential impact of ganglioside antibodies on axonal degeneration and neuronal loss in limbic encephalitis pends to be further investigated.

show abstract

Section: Discussionsupporting

confidence: 80%

Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis

Gürsoy¹,

Selçuk²,

Tunc³

et al. 2021

Turk J Med Sci

View full text Add to dashboard Cite

show abstract

“…7,[10][11][12][13][14] on the other hand, they have recently been found to be highly important in immunology, as target antigens in several auto-immune neurological disorders. 15,16 Different modern directions of investigation provided data supporting the pathogenic role of anti-GG antibodies in several immune neuropathies. 17,18 Structural elucidation of individual GG components in mixtures extracted from the human brain represents a fundamental requirement for the determination of their composition in defined brain regions and for correlating the specificity of these structures with the specialized function of each area of the cNS in health and disease.…”

mentioning

confidence: 99%

High-Throughput Analysis of Gangliosides in Defined Regions of Fetal Brain by Fully Automated Chip-Based Nanoelectrospray Ionization Multi-Stage Mass Spectrometry

Serb

Schiopu

Flangea

et al. 2009

Eur J Mass Spectrom (Chichester)

View full text Add to dashboard Cite

Gangliosides (GGs), a large group of sialylated glycosphingolipids, are considered biomarkers of human brain development, aging and certain diseases. Determination of individual GG components in complex mixtures extracted from a human brain represents a fundamental prerequisite for correlating their specificity with the specialized function of each brain area. In the context of modern glycomics, detailed investigation of GG expression and structure in human brain requires a continuous development and application of innovative methods able to improve the quality of data and speed of analysis. In this work, for the first time, a high-throughput mapping and sequencing of gangliosides in human fetal brain was performed by a novel mass spectrometry (MS)-based approach developed recently in our laboratory. Three GG mixtures extracted and purified from different regions of the same fetal brain in the 36th gestational week: frontal neocortex (NEO36), white matter of the frontal lobe (FL36) and white matter of the occipital lobe (OL36) were subjected to comparative high-throughput screening and multi-stage fragmentation by fully automated chip-based nanoelectrospray ionization (nanoESI) high capacity ion trap (HCT) MS. Using this method, in only a few minutes of signal acquisitions, over 100 GG and asialo-GG species were detected and identified in the three mixtures. Obtained data revealed for the first time that differences in GG expression in human fetal brain are dependent on phylogenetic development rather than topographic factors. While a significant variation of GG distribution in NEO36 vs FL36 was observed, no significant differences in GG expression in white matter of frontal vs occipital lobe were detected. Additionally, the largest number of species was identified in NEO36, which correlates with the functional complexity of neocortex as the newest brain region. In the last stage of analysis, using MS2–MS3 molecular ion fragmentation at variable amplitudes, a NEO36-associated GD1b isomer could clearly be discriminated. Present results indicate that the combination of fully automated chipESI with HCT MS n is able to provide ultra-fast, sensitive and reliable analyses of complex lipid-linked carbohydrates from which the pattern of their expression and structure in a certain type of bio-matrix can be determined.

show abstract

“…Just a few cases of myoclonus associated with anti‐GQ1b antibody have been reported . Those cases showed opsoclonus with concomitant polymyoclonus after an upper respiratory infection.…”

Section: Discussionmentioning

confidence: 99%

Miller Fisher syndrome associated with polymyoclonus

Inoue¹,

Yamamoto²,

Tsuzaki³

et al. 2015

Neurology & Clinical Neurosc

View full text Add to dashboard Cite

We described a 35-year-old man who presented with diplopia, dysarthria, loss of deep tendon reflexes, and rapidly progressive unsteadiness in standing and walking. Two days after admission, frequent, irregular, and shock-like movements appeared in the hands and abdomen. On laboratory examination, serum immunoglobulin G antibodies against GQ1b, GT1a and GD1b were all positive. Electrophysiologically, recording of the involuntary movements with surface electromyography showed irregular sharp discharges especially during weak muscle contraction. The clinical diagnosis of Miller Fisher syndrome associated with polymyoclonus was made, and all neurological symptoms including myoclonus improved after intravenous injection of immunoglobulin. The distribution of myoclonus and electrophysiological data suggested that the myoclonus, though different from propriospinal or spinal segmental myoclonus in the distribution and propagation, might still be of spinal cord origin.

show abstract

Ocular Flutter, Generalized Myoclonus, and Trunk Ataxia Associated With Anti-GQ1b Antibodies

Cited by 36 publications

References 12 publications

Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis

Serum antiganglioside antibodies in patients with autoimmune limbic encephalitis

High-Throughput Analysis of Gangliosides in Defined Regions of Fetal Brain by Fully Automated Chip-Based Nanoelectrospray Ionization Multi-Stage Mass Spectrometry

Miller Fisher syndrome associated with polymyoclonus

Contact Info

Product

Resources

About