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.
Highlights
Since the discovery of anti-GQ1b antibody in typical Miller Fisher syndrome (MFS) in 1992 [1], Bickerstaff brainstem encephalitis (BBE), acute ophthalmoplegia (AO), and other variants of MFS and Guillain-Barré syndrome (GBS) have been associated with anti-GQ1b antibody [2]
All diagnoses met the criteria of anti-GQ1b antibody syndrome proposed by Wakerley et al.: a continuous spectrum disease includs MFS, GBS, BBE, and acute ophthalmoparesis et al characterized by positive anti-GQ1b immunoglobulin G (IgG) in serum [2, 3, 9]
In order to dinineate the comprehensive picture of pediatric anti-GQ1b antibody syndrome, the clinical data of all the 12 individuals were combined with the published study to be analyzed systematically
Summary
Since the discovery of anti-GQ1b antibody in typical Miller Fisher syndrome (MFS) in 1992 [1], Bickerstaff brainstem encephalitis (BBE), acute ophthalmoplegia (AO), and other variants of MFS and Guillain-Barré syndrome (GBS) have been associated with anti-GQ1b antibody [2]. As MFS, GBS, BBE, and AO are closely related and form a continuous range, as well as the serum-positive anti-GQ1b antibody status among the aforementioned syndromes, a more inclusive nomenclature, i.e., “anti-GQ1b antibody syndrome” has been proposed to include the common serological profile when referring to the clinical syndromes described by both Bickerstaff and Fisher [2, 3]. The phenotypes of anti-GQ1b antibody syndrome have been expanded to acute post-infectious ophthalmoplegia without ataxia (atypical MFS) [4], ataxic GBS [5], pharyngeal–cervical– brachial weakness (PCBW) [6], and other subtypes [7]. A thorough reevaluation of the subtypes of anti-GQ1b antibody syndrome in childhood under the new framework is essential
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