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Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome

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Abstract

Background & purpose

In this retrospective study, we aimed at defining the clinical, paraclinical and outcome features of acute neurological syndromes associated with anti-GQ1b antibodies.

Results

We identified 166 patients with neurological symptoms appearing in less than 1 month and anti-GQ1b antibodies in serum between 2012 and 2022. Half were female (51%), mean age was 50 years (4–90), and the most frequent clinical features were areflexia (80% of patients), distal upper and lower limbs sensory symptoms (78%), ophthalmoplegia (68%), sensory ataxia (67%), limb muscle weakness (45%) and bulbar weakness (45%). Fifty-three patients (32%) presented with complete (21%) and incomplete (11%) Miller Fisher syndrome (MFS), thirty-six (22%) with Guillain–Barre syndrome (GBS), one (0.6%) with Bickerstaff encephalitis (BE), and seventy-three (44%) with mixed MFS, GBS & BE clinical features. Nerve conduction studies were normal in 46% of cases, showed demyelination in 28%, and axonal loss in 23%. Anti-GT1a antibodies were found in 56% of cases, increased cerebrospinal fluid protein content in 24%, and Campylobacter jejuni infection in 7%. Most patients (83%) were treated with intravenous immunoglobulins, and neurological recovery was complete in 69% of cases at 1 year follow-up. One patient died, and 15% of patients relapsed. Age > 70 years, initial Intensive Care Unit (ICU) admission, and absent anti-GQ1b IgG antibodies were predictors of incomplete recovery at 12 months. No predictors of relapse were identified.

Conclusion

This study from Western Europe shows acute anti-GQ1b antibody syndrome presents with a large clinical phenotype, a good outcome in 2/3 of cases, and frequent relapses.
Title
Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome
Authors
Martin Coly
David Adams
Shahram Attarian
Françoise Bouhour
Jean-Philippe Camdessanché
Guillaume Carey
Cécile Cauquil
Jean-Baptiste Chanson
Pascale Chrétien
Alain Créange
Emilien Delmont
Guillaume Fargeot
Simon Frachet
Thierry Gendre
Thierry Kuntzer
Céline Labeyrie
Thierry Maisonobe
Maud Michaud
Maximilien Moulin
Guillaume Nicolas
Jean-Baptiste Noury
Yann Péréon
Angela Puma
Guilhem Sole
Frédéric Taithe
Céline Tard
Marie Théaudin
Serge Timsit
Laura Venditti
Andoni Echaniz-Laguna
Publication date
20-05-2024
Publisher
Springer Berlin Heidelberg
Published in
Journal of Neurology / Issue 8/2024
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI
https://doi.org/10.1007/s00415-024-12410-4
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