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

期刊论文
发表日期：

2024年5月20日

查看全文

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

摘要

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.