Acute Isolated External Ophthalmoplegia: Think of Anti-GQ1b Antibody Syndrome

Neurol India. 2022 Sep-Oct;70(5):2159-2162. doi: 10.4103/0028-3886.359274.


Acute-onset ophthalmoplegia is a perplexing diagnosis in a young child. When the full-blown picture of ophthalmoplegia, ataxia, and areflexia is evident, the diagnosis of Miller-Fisher syndrome (MFS), a variant of Guillain-Barre syndrome (GBS), is almost certain. However, the same is not true for isolated external ophthalmoplegia as it is etiologically heterogeneous. Only anecdotal case reports of childhood-onset acute ophthalmoplegia exist in the literature. Adult series suggest that acute onset external ophthalmoplegia is often immune-mediated and is secondary to anti-GQ1b antibodies. We present a 30-month-old boy with acute-onset bilateral external ophthalmoplegia with highly elevated serum anti-GQ1b antibodies. The child had a rapid and complete recovery with intravenous immunoglobulin. A review of all published cases of childhood anti-GQ1b antibody syndrome was performed. The case highlights that anti-GQ1b antibody syndrome should be considered even in young children with acute-onset external ophthalmoplegia. The disease has a favorable prognosis. The majority improve on conservative management. Treatment with steroids or IVIG may be considered in some after weighing the risks and benefits.

Keywords: Acute ophthalmoplegia without ataxia; Guillain–Barre syndrome; IVIG; Miller–Fisher syndrome; anti-GQ1b syndrome; total external ophthalmoplegia.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Adult
  • Ataxia / diagnosis
  • Child
  • Child, Preschool
  • Gangliosides
  • Guillain-Barre Syndrome* / complications
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Male
  • Miller Fisher Syndrome* / complications
  • Miller Fisher Syndrome* / diagnosis
  • Ophthalmoplegia* / complications
  • Ophthalmoplegia* / etiology


  • Gangliosides
  • Immunoglobulins, Intravenous