Elsevier

Pediatric Neurology

Volume 41, Issue 6, December 2009, Pages 451-452
Pediatric Neurology

Case Report
Isolated Ptosis as Acute Ophthalmoplegia Without Ataxia, Positive for Anti-GQ1b Immunoglobulin G

https://doi.org/10.1016/j.pediatrneurol.2009.07.008Get rights and content

Anti-GQ1b IgG antibody syndrome comprises a wide range of diseases presenting with ophthalmoplegia and ataxia. Anti-GQ1b antibodies have been strongly associated in the literature with Miller Fisher Syndrome, with acute ophthalmoplegia associated with Guillain-Barré syndrome, and with isolated ophthalmoplegia. Acute ophthalmoplegia presents as various combinations of external and internal ophthalmoplegia. Reported here is a novel case of isolated ptosis as a manifestation of ophthalmoplegia. The present finding of bilateral ptosis and areflexia with anti-GQ1b IgG antibody positivity helps confirm the existence of the syndrome. Further research is needed on diagnosis and treatment.

Introduction

Anti-GQ1b antibodies have been strongly associated in the literature with Miller Fisher Syndrome [1], with acute ophthalmoplegia associated with Guillain-Barré, and with isolated ophthalmoplegia [2], [3]. Anti-GQ1b IgG antibody syndrome comprises a wide range of disorders presenting with ophthalmoplegia and ataxia [4]. Acute ophthalmoplegia associated with anti-GQ1b antibody manifests as various combinations of external and internal ophthalmoplegia, including ptosis [3]. Reported here is the case of a patient with anti-GQ1b antibody positivity who had bilateral ptosis as the only manifestation of acute ophthalmoplegia and areflexia. The case falls within the spectrum of anti-GQ1b IgG antibody syndrome illnesses.

Section snippets

Case Report

A 2-year-old boy presented with a history of fever and cough for 6-7 days. He had had a similar episode a year previously, for which he was given salbutamol nebulizations. On examination, he was found to be febrile and tachypneic, with diffuse rhonchi on chest auscultation. Investigations revealed a total leukocyte count of 10,000/mm3 with 70% neutrophils, 27% lymphocytes, and 1% eosinophils; blood cultures were found to be sterile. Chest radiography revealed bilateral hyperinflation with

Discussion

Miller Fisher syndrome, which is characterized by ophthalmoplegia, ataxia, and areflexia [5], has been strongly associated with anti-GQ1b IgG antibodies [1], [2], [6]. Motor strength is usually preserved. Anti-GQ1b IgG antibody has also been found in patients with Guillain-Barré syndrome who had ophthalmoparesis. Also, anti-GQ1b IgG antibody has also been closely associated with Bickerstaff's brain stem encephalitis, a clinical entity described in patients with acute onset of external

References (6)

  • N. Yuki et al.

    Frequent presence of anti-GQ1b antibody in Fisher's syndrome

    Neurology

    (1993)
  • A. Chiba et al.

    Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies

    Neurology

    (1993)
  • S.H. Lee et al.

    Acute ophthalmoplegia (without ataxia) associated with anti-GQ1b antibody

    Neurology

    (2008)
There are more references available in the full text version of this article.

Cited by (0)

View full text