Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome
2 SU - Sorbonne Université
3 Université Paris-Saclay
4 DHNS - Maladies et hormones du système nerveux
5 TIMONE - Hôpital de la Timone [CHU - APHM]
6 Hôpital neurologique et neurochirurgical Pierre Wertheimer [CHU - HCL]
7 CHU ST-E - Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne]
8 CHRU Lille - Centre Hospitalier Régional Universitaire [CHU Lille]
9 LilNCog - Lille Neurosciences & Cognition - U 1172
10 HUS - Hôpitaux Universitaires de Strasbourg
11 CNRS - Centre National de la Recherche Scientifique
12 INSERM - Institut National de la Santé et de la Recherche Médicale
13 UTCBS - UM 4 (UMR 8258 / U1267) - Unité de Technologies Chimiques et Biologiques pour la Santé
14 Hôpital Henri Mondor
15 ENT - Excitabilité nerveuse et thérapeutique
16 NEURIT - Neuropathies et Innovations Thérapeutiques
17 NNerf - Centre de référence national neuropathies périphériques rares [CHU Limoges]
18 CHUV - Centre Hospitalier Universitaire Vaudois = Lausanne University Hospital [Lausanne]
19 UNIL - Université de Lausanne = University of Lausanne
20 CHU Pitié-Salpêtrière [AP-HP]
21 Service de neurologie [CHRU Nancy]
22 Service de neurologie [Reims]
23 Hôpital Raymond Poincaré [AP-HP]
24 UVSQ Santé - UFR Sciences de la santé Simone Veil
25 LBAI - Lymphocytes B, Autoimmunité et Immunothérapies
26 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
27 CHU Nice [Cimiez]
28 CHU Bordeaux - Centre Hospitalier Universitaire de Bordeaux
29 CHU Gabriel Montpied [Clermont-Ferrand]
- Fonction : Auteur
- PersonId : 1134581
- ORCID : 0000-0003-1012-9783
- Fonction : Auteur
- PersonId : 1333393
- ORCID : 0000-0003-4221-3840
- Fonction : Auteur
- PersonId : 1435943
- ORCID : 0000-0003-3838-1622
- Fonction : Auteur
- PersonId : 1383498
- ORCID : 0000-0002-9758-0085
Résumé
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 (4u201390), 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 Guillainu2013Barre 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.