%0 Journal Article
%T Clinical, paraclinical and outcome features of 166 patients with acute anti-GQ1b antibody syndrome.
%A Coly M
%A Adams D
%A Attarian S
%A Bouhour F
%A Camdessanché JP
%A Carey G
%A Cauquil C
%A Chanson JB
%A Chrétien P
%A Créange A
%A Delmont E
%A Fargeot G
%A Frachet S
%A Gendre T
%A Kuntzer T
%A Labeyrie C
%A Maisonobe T
%A Michaud M
%A Moulin M
%A Nicolas G
%A Noury JB
%A Péréon Y
%A Puma A
%A Sole G
%A Taithe F
%A Tard C
%A Théaudin M
%A Timsit S
%A Venditti L
%A Echaniz-Laguna A
%J J Neurol
%V 271
%N 8
%D 2024 Aug 20
%M 38767661
%F 6.682
%R 10.1007/s00415-024-12410-4
%X OBJECTIVE: 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.
CONCLUSIONS: 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.