Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome.
Détails
ID Serval
serval:BIB_D71C583FE768
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome.
Périodique
Journal of neurology
ISSN
1432-1459 (Electronic)
ISSN-L
0340-5354
Statut éditorial
Publié
Date de publication
08/2024
Peer-reviewed
Oui
Volume
271
Numéro
8
Pages
4982-4990
Langue
anglais
Notes
Publication types: Journal Article
Publication Status: ppublish
Publication Status: ppublish
Résumé
In this retrospective study, we aimed at defining the clinical, paraclinical and outcome features of acute neurological syndromes associated with anti-GQ1b antibodies.
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.
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.
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.
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.
Mots-clé
Humans, Female, Male, Middle Aged, Adult, Gangliosides/immunology, Aged, Retrospective Studies, Young Adult, Adolescent, Autoantibodies/blood, Autoantibodies/cerebrospinal fluid, Aged, 80 and over, Miller Fisher Syndrome/physiopathology, Miller Fisher Syndrome/blood, Miller Fisher Syndrome/diagnosis, Child, Child, Preschool, Guillain-Barre Syndrome/blood, Guillain-Barre Syndrome/physiopathology, Guillain-Barre Syndrome/diagnosis, Guillain-Barre Syndrome/immunology, Anti-GQ1b antibodies, Bickerstaff’s brainstem encephalitis, Guillain–Barre syndrome, Miller-Fisher syndrome
Pubmed
Web of science
Création de la notice
24/05/2024 9:41
Dernière modification de la notice
20/08/2024 7:22