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Incidence and Ocular Features of Pediatric Myasthenias
Sasha A Mansukhani1, Erick D Bothun1, Nancy N Diehl2
1Department of Ophthalmology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA.
Insights
Myasthenia gravis in children is rare, with juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) being the most common forms. Both often present with ocular symptoms, and JMG shows a higher likelihood of improvement.
Area of Science:
- Neurology
- Pediatrics
- Ophthalmology
Background:
- Myasthenia gravis (MG) in children is a rare neuromuscular disorder.
- Two predominant forms exist: juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS).
- Understanding the incidence, demographics, and clinical features of pediatric MG is crucial for diagnosis and management.
Purpose of the Study:
- To determine the incidence and demographics of myasthenia in children.
- To describe the ocular findings in pediatric myasthenia patients.
- To analyze the outcomes and response to treatment in different forms of childhood MG.
Main Methods:
- A retrospective cohort study was conducted.
- Medical records of children (<19 years) diagnosed with any form of myasthenia at Mayo Clinic between 1966 and 2015 were reviewed.
- Incidence, diagnostic age, subtypes, ocular involvement, and treatment outcomes were analyzed.
Main Results:
- The age- and sex-adjusted incidence of pediatric MG was 0.35 per 100,000 children.
- Juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) constituted the majority of cases.
- Ocular involvement, including ptosis and ocular movement deficits, was common in both JMG (90.3%) and CMS (85.1%).
- Improvement was observed in 88.8% of JMG patients and 58.3% of CMS patients.
Conclusions:
- Congenital myasthenic syndrome (CMS) and juvenile myasthenia gravis (JMG) are the primary forms of myasthenia in children.
- Ocular manifestations are frequently observed in both JMG and CMS.
- Children with JMG demonstrate a higher rate of improvement compared to those with CMS.
Purpose:
To report the incidence, demographics, and ocular findings of children with myasthenia.
Design:
Retrospective cohort study.
Methods:
The medical records of all children (<19 years) examined at Mayo Clinic with any form of myasthenia from January 1 1966, through December 31, 2015, were retrospectively reviewed.
Results:
A total of 364 children were evaluated during the study period, of which 6 children were residents of the Olmsted County at the time of their diagnosis, yielding an annual age- and sex-adjusted incidence of 0.35 per 100 000 <19 years, or 1 in 285 714 <19 years. The incidence of juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) was 0.12 and 0.23 per 100 000, respectively. Of the 364 study children, 217 (59.6%) had JMG, 141 (38.7%) had CMS, and 6 (1.7%) had Lambert-Eaton syndrome, diagnosed at a median age of 13.5, 5.1, and 12.6 years, respectively. A majority of the JMG and CMS patients had ocular involvement (90.3% and 85.1%, respectively), including ptosis and ocular movement deficits. Among children with at least 1 year of follow-up (JMG; median, 7.1 years, CMS; median, 7.0 years), improvement was seen in 88.8% of JMG patients (complete remission in 31.3%) and in 58.3% of CMS patients.
Conclusion:
Although relatively rare, myasthenia gravis in children has 2 predominant forms, CMS and JMG, both of which commonly have ocular involvement. Improvement is more likely in children with the juvenile form.
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