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Demographics and ocular findings in children with myasthenia

Natalia Arruti1, Pinki Munot2, Richard Bowman3,4

  • 1Clinical and Academic Department of Ophthalmology, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London, WC1N 3JH, UK. n.arruti@nhs.net.

Eye (London, England)
|March 26, 2022
PubMed

Insights

Juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) are key pediatric neuromuscular disorders. Ocular symptoms like ptosis are common, but most children improve with treatment.

Area of Science:

  • Neurology
  • Ophthalmology
  • Pediatrics

Background:

  • Myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular junctions.
  • Pediatric MG encompasses juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS).
  • Understanding the demographics and clinical features of pediatric MG is crucial for diagnosis and management.

Purpose of the Study:

  • To characterize the demographics of pediatric myasthenia gravis patients.
  • To identify the ocular features and common presentations of myasthenia gravis in children.
  • To analyze treatment outcomes in pediatric MG.

Main Methods:

  • Retrospective chart review of pediatric patients diagnosed with myasthenia gravis.
  • Inclusion criteria: patients under 18 years old seen at Great Ormond Street Hospital from 2016-2020.
  • Data collected included demographics, clinical presentation, diagnostic findings, and treatment response.

Main Results:

  • 49 children were analyzed, with a female predominance.
  • Juvenile myasthenia gravis (JMG) was more common than congenital myasthenic syndrome (CMS).
  • Ocular involvement, particularly ptosis, was frequent (49%), especially in JMG (70.4%).
  • Pyridostigmine was the primary treatment, with 73.5% of patients showing improvement.

Conclusions:

  • Juvenile myasthenia gravis (JMG) is the predominant form of pediatric MG.
  • Ocular manifestations, including ptosis, are significant presenting signs in pediatric MG.
  • Medical management, particularly with pyridostigmine, leads to favorable outcomes in the majority of pediatric MG patients.
Abstract

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