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Incidence and Ocular Features of Pediatric Myasthenias.

Authors :
Mansukhani SA
Bothun ED
Diehl NN
Mohney BG
Source :
American journal of ophthalmology [Am J Ophthalmol] 2019 Apr; Vol. 200, pp. 242-249. Date of Electronic Publication: 2019 Jan 14.
Publication Year :
2019

Abstract

Purpose: To report the incidence, demographics, and ocular findings of children with myasthenia.<br />Design: Retrospective cohort study.<br />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.<br />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.<br />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.<br /> (Copyright © 2019 Elsevier Inc. All rights reserved.)

Details

Language :
English
ISSN :
1879-1891
Volume :
200
Database :
MEDLINE
Journal :
American journal of ophthalmology
Publication Type :
Academic Journal
Accession number :
30653958
Full Text :
https://doi.org/10.1016/j.ajo.2019.01.004