Response to treatment in pediatric ocular myasthenia gravis

Muscle Nerve. 2020 Feb;61(2):226-230. doi: 10.1002/mus.26745. Epub 2019 Nov 7.

Abstract

Introduction: Juvenile myasthenia gravis (JMG), a pediatric autoimmune neuromuscular junction disorder, includes generalized (GMG), and ocular (OMG) variants. We sought to determine whether differences existed between OMG and GMG children regarding demographics or treatment response.

Methods: We performed retrospective analysis of 60 children with JMG seen between 1990 and 2018. Osserman scores were used to define OMG and GMG. The myasthenia scale of Millichap and Dodge was used to assess treatment responses.

Results: There were no differences between GMG and OMG regarding time interval from disease onset to prednisone initiation (P = .42), or treatment response according to Millichap and Dodge (P = .12). Compared with GMG, OMG children showed younger age of disease onset and better outcomes after treatment. No OMG patients progressed to generalized disease during the follow-up period.

Discussion: Compared with GMG, OMG patients had earlier disease onset and improved outcomes after treatment.

Keywords: juvenile myasthenia gravis; myasthenia gravis; neuroimmunological disorder; ocular myasthenia gravis; pediatric myasthenia gravis; response to treatment.

MeSH terms

  • Adolescent
  • Age of Onset
  • Anti-Inflammatory Agents / therapeutic use
  • Child
  • Child, Preschool
  • Disease Progression
  • Female
  • Humans
  • Infant
  • Male
  • Myasthenia Gravis / drug therapy*
  • Myasthenia Gravis / physiopathology
  • Oculomotor Muscles / physiopathology
  • Prednisone / therapeutic use
  • Retrospective Studies
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Prednisone