%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.