Clinical Characterization of Anti-GQ1b Antibody Syndrome in Childhood

Abstract

Objective: To delineate the comprehensive clinical features of anti-GQ1b antibody syndrome in childhood. Methods: The clinical data of children diagnosed with anti-GQ1b antibody syndrome at two Chinese tertiary pediatric neurology centers were collected and analyzed. We also conducted a systematic literature review on anti-GQ1b antibody syndrome in children. Results: This study included 78 children with anti-GQ1b antibody syndrome, consisting of 12 previously unreported cases from the two Chinese centers. The median onset age was 10 years (range, 2-18 years). The most common phenotype was acute ophthalmoparesis (32%), followed by classic Miller Fisher syndrome (15%), and Bickerstaff brainstem encephalitis (12%). External ophthalmoplegia (48%), sensory disturbance (9%), and bulbar palsy (9%) were the three most frequent onset symptom manifestations. Brain or spinal lesions on MRI and abnormal recordings by nerve conduction study were present in 18% (12/68) and 60% (27/45) of cases, respectively. There was CSF albuminocytologic dissociation in 34% of the patients (23/68). IV immunoglobulin alone or combined with steroids or plasma exchange was administered to 58% of patients (42/72). We did not find a significant correlation between early improvement up to 3 months and age onset and phenotype. All patients showed different degrees of recovery, and 81% (57/70) had complete recovery within 1 year. Conclusions: Acute ophthalmoparesis and classic Miller Fisher syndrome are the most common phenotypes of anti-GQ1b antibody syndrome in childhood. The majority of patients show good response to immunotherapy and have favorable prognosis.

Keywords: Guillain-Barré syndrome; Miller Fisher syndrome; anti-GQ1b antibody; diangnosis; immunotherapy; pediatric.

Figure 1

Algorithm of systematic review.

Figure 2

The onset symptoms and phenotypes of this cohort. (A) Phenotypes of anti-GQ1b antibody syndrome; (B) Subtypes categorized by age; (C) Onset symptoms of anti-GQ1b antibody syndrome in childhood; (D) Onset symptoms categorized by age. AAH, acute ataxic hypersomnolence; AAN, acute ataxic neuropathy; AC, altered consciousness; AO, acute ophthalmoparesis; AP, acute ptosis; APW, acute pharyngeal weakness; BBE, Bickerstaff brainstem encephalitis; BP, bulbar palsy; BWP, bifacial weakness with paraesthesias; EO, external ophthalmoplegia; FP, facial paralysis; GBS, Guillain-Barré syndrome; IO, internal ophthalmoplegia; LW, limb weakness; MFS, Miller Fisher syndrome; NA, not available; PCBW, pharyngeal-cervical-brachial weakness; PGBS, paraparetic GBS; SD, sensory disturbance.

Figure 3

Correlation between ACD and NCS. Ab, anti-GQ1b antibodies; ACD, albuminocytologic dissociation; NCS, nerve conduction study.