Anti-GQ1b antibody syndrome presenting as acute isolated bilateral ophthalmoplegia: Report on two patients and review of the literature

Eur J Paediatr Neurol. 2016 May;20(3):439-43. doi: 10.1016/j.ejpn.2016.02.002. Epub 2016 Feb 18.

Abstract

Background: Miller Fisher syndrome (MFS) is an acute polyradiculoneuritis regarded as an uncommon clinical variant of Guillain-Barré syndrome (GBS). MFS is characterized by the acute onset of the clinical triad of ophthalmoplegia, cereballar ataxia and areflexia. Atypical forms of MFS presenting as isolated ophthalmoplegia without ataxia have been rarely described, mostly in adults.

Patients: We present two cases of acute isolated bilateral ophthalmoplegia in childhood, both occurring shortly after Campylobacter jejuni enteritis. Serum analysis of anti-ganglioside antibodies revealed elevated levels of anti-GQ1b IgG and IgM. We observed in both children complete spontaneous resolution several weeks after onset.

Conclusion: The cases of the two patients confirm the rare but possible occurrence of atypical MFS in young children a few weeks after gastrointestinal infection. Identification of high levels of anti-GQ1b antibodies in the serum may help confirm the diagnosis of MFS even when its clinical presentation is incomplete.

Keywords: Acute ophthalmoplegia; Anti-GQ1b antibody; Atypical Miller Fisher syndrome; Children.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acute Disease
  • Antibodies / blood
  • Campylobacter Infections / complications
  • Campylobacter jejuni
  • Child
  • Child, Preschool
  • Gangliosides / immunology*
  • Gastroenteritis / complications
  • Humans
  • Male
  • Miller Fisher Syndrome / complications*
  • Miller Fisher Syndrome / diagnosis*
  • Ophthalmoplegia / etiology*

Substances

  • Antibodies
  • Gangliosides
  • GQ1b ganglioside