Abstract

Background: We aimed to investigate the characteristic presentation of Miller Fisher syndrome (MFS) in pediatrics and compare it with that in adults. Methods: We performed a retrospective review of medical records, laboratory findings, and disease course of pediatric MFS. The data were compared with those of adult MFS, and literature review was done. Unpaired and paired comparisons between groups were made using Wilcoxon rank-sum and signed-rank tests, respectively. Results: Median age for pediatric MFS was 9.8 ± 6.5 years. There were 5 (45.5%) male and 6 (54.5%) female patients. All patients had preceding infection. Two patients (22.2%) had tested positive for anti-GQ1b antibody. Ten patients (90.1%) were treated with intravenous immunoglobulin, and 2 (18.2%) also received intravenous methylprednisolone. Within one month, 8 (72.7%) patients showed recovery, and all 11 (100%) recovered fully within 3 months. Further, the pediatric group had higher frequency of unilateral involvement of ophthalmoplegia, ataxia, and autonomic symptoms but lower antiganglioside antibody positivity and manifestations of areflexia than the adult group. Conclusions: Neuro-ophthalmic manifestations and disease course of pediatric MFS were similar to those of adult MFS as stated in the literature. However, the presence of autonomic symptoms was higher and anti-GQ1b antibody positivity was lower in pediatric MFS than in adult MFS.

Highlights

  • Miller Fisher syndrome (MFS) is an acute self-limiting disorder characterized by a clinical triad of ophthalmoplegia, ataxia, and areflexia [1,2]

  • Unilateral involvement at disease onset. b Statistics using the number of patients who were available for each test. c GM, GD, GT antibody. d Only corticosteroid treatment. This retrospective comparative study showed that neuro-ophthalmic manifestations and disease course of pediatric MFS were similar to those of adult MFS

  • There was a lower incidence of bilateral ocular manifestation at initial presentation among pediatric MFS patients than among adult MFS patients

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Summary

Introduction

Miller Fisher syndrome (MFS) is an acute self-limiting disorder characterized by a clinical triad of ophthalmoplegia, ataxia, and areflexia [1,2]. Anti-GQ1b antibodies have been considered as a crucial biomarker for MFS. These clinical findings and laboratory results were based on the adult patient group because the prevalence of MFS in pediatric patients is significantly lower than that in adults [6,7]. Two patients (22.2%) had tested positive for anti-GQ1b antibody. The pediatric group had higher frequency of unilateral involvement of ophthalmoplegia, ataxia, and autonomic symptoms but lower antiganglioside antibody positivity and manifestations of areflexia than the adult group. The presence of autonomic symptoms was higher and anti-GQ1b antibody positivity was lower in pediatric MFS than in adult MFS

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