Varied antibody reactivities and clinical relevance in anti-GQ1b antibody–related diseases

Abstract

ObjectiveTo investigate the relationship between antibody reactivities against glycolipid complexes and clinical features in Miller Fisher syndrome (MFS), Bickerstaff brainstem encephalitis (BBE), and Guillain-Barré syndrome with ophthalmoplegia (GBS-OP).MethodsUsing glycoarray, antibodies against 10 glycolipid antigens (GM1, GM2, GM4, GD1a, GD1b, GQ1b, galactocerebroside, lactosylceramide, GA1, and sulfatide) and 45 glycolipid complexes consisting 2 of the glycolipids were examined in the sera of 63 patients with GBS-OP, 37 patients with MFS, and 27 patients with BBE.ResultsAntibodies to antigens containing GQ1b were identified in 73% of patients with GBS-OP (46/63), 86.5% of patients with MFS (32/37), and 74.1% of patients with BBE (20/27), and GD1b-related antibodies were identified in 49.2% of patients with GBS-OP (31/63), 29.7% of patients with MFS (11/37), and 11.1% of patients with BBE (3/27). Comparing clinical features between patients with GBS-OP with and without both antibodies, the proportion of patients requiring artificial ventilation and presenting moderate or severe muscle weakness was higher in the positive group than in the negative group (p = 0.017 and p = 0.046, respectively).ConclusionsAntibodies binding to antigens containing GD1b and to those containing GQ1b may be involved in the development of limb weakness and respiratory failure in anti-GQ1b antibody–related diseases.

Guillain–Barré syndrome spectrum associated with COVID-19: an up-to-date systematic review of 73 cases

“…Regarding the distribution of GBS electrophysiological variants, our analysis showed that COVID-19-associated GBS manifests prevalently with AIDP and, to a lesser extent, with AMSAN and AMAN, in line with classic GBS in Western countries [ 66 , 85 ]. Conversely, the observation of positive anti-GD1b antibodies in one COVID-19-related MFS patient and negative anti-ganglioside antibodies in other five cases appear in discordance with the high prevalence (≈ 90%) of anti-GQ1b antibodies among non-COVID-19 MFS cases [ 86 ], and may suggest different immune-mediated mechanisms. However, these results could not be generalized until a wider population would be tested.…”

Immune-mediated neurological syndromes in SARS-CoV-2-infected patients

“…GD1b is one of the most represented gangliosides in nerve tissues, but has been less studied than its GQ1b and GM1 counterparts [ 10 ]. Anti-GD1b antibodies have been described in the clinical spectrum of Guillain–Barré syndrome, Miller Fisher syndrome and Bickerstaff brainstem encephalitis, and are associated with more severe disease and slower recovery [ 11 , 12 ]. More recently, anti-GD1b IgG antibodies were reported in a COVID-19 patient with Miller Fisher syndrome [ 3 ].…”

Guillain–Barre Syndrome

“…33,34 Antibodies to GQ1b gangliosides are found to be highly prevalent in MFS, BBE, and in GBS with ophthalmoplegia. 35 The high abundance of GQ1b gangliosides in extraocular neuromuscular junctions, muscle spindles, dorsal root ganglia, and possibly lower brainstem structures (reticular formation and vagal nerve) can explain the typical clinical presentation of these syndromes, [36][37][38] while the presence of other antibodies (such as GM1, GM1b, GD1a, or GalNAc-GD1antibodies) further explains the possible variability in clinical spectrum and overlap with other GBS variants. 38 A summary of the associations between GBS clinical presentations and different antibodies can be found in ►Table 1.…”