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Childhood ocular myasthenia gravis
Jong-Hyun Kim1, Jeong-Min Hwang, Yong Seung Hwang
1Department of Ophthalmology, Seoul National University College of Medicine, Seoul, South Korea.
Insights
Ocular myasthenia gravis (MG) in children frequently causes ptosis and strabismus, with amblyopia also noted. Eye movement limitations were less responsive to treatment than ptosis.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Ocular myasthenia gravis (MG) is a rare autoimmune disorder affecting neuromuscular transmission.
- Clinical manifestations in pediatric ocular MG are not well-documented.
Purpose of the Study:
- To investigate the clinical manifestations and ocular findings in children with ocular MG.
- To report on findings rarely documented in existing literature.
Main Methods:
- Retrospective case series of 24 pediatric patients (<15 years) with ocular MG.
- Reviewed medical records for clinical and ocular findings.
- Utilized tests including alternate prism cover, ductions, versions, and visual acuity.
Main Results:
- High incidence of ptosis (96%) and strabismus (88%), predominantly exotropia with vertical heterotropia.
- Limited ductions observed in 71% of patients, more commonly lateral rectus underaction.
- Amblyopia reported in 21% of cases; ptosis responded better to treatment than motility limitations.
Conclusions:
- Pediatric ocular MG presents with significant ptosis and strabismus.
- Amblyopia is a notable finding in this population.
- Limited ocular motility shows relative nonresponsiveness to treatment compared to ptosis.
Purpose:
To investigate the clinical manifestations and ocular findings in children with ocular myasthenia gravis (MG) that rarely have been reported in the literature.
Design:
Retrospective, noncomparative case series.
Participants:
Twenty-four consecutive patients less than 15 years of age with ocular MG treated between June 1988 and July 2001.
Methods:
The medical records of 6 boys and 18 girls with ocular MG were reviewed retrospectively.
Main Outcome Measures:
Alternate prism cover and uncover test, examination of ductions and versions, and visual acuity.
Results:
Mean age at onset was 38 months. Ptosis was found in 23 patients (96%), strabismus in 21 patients (88%), and amblyopia in 5 patients (21%). Exotropia combined with vertical heterotropia was the most frequent type of strabismus. Ductions were limited in 17 patients (71%), among whom supraduction or infraduction limitations were most frequently observed. Contrary to previous reports, medial rectus underaction was less common than lateral rectus underaction. Manifestations of strabismus and limitation of duction were variable and changed frequently during the follow-up period. The combined use of prednisone and pyridostigmine was found to be the predominant form of maintenance therapy, and ptosis was more responsive to drug therapy than limited ocular motility.
Conclusions:
Children with ocular MG were found to have a high incidence of ptosis (96%) and exotropia and vertical hyperdeviation. Limitation on adduction was less common than that on abduction. First reported incidence of amblyopia (21%) and a relative nonresponsiveness of the limitation of eye movement to treatment were also noted.
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