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Long-term outcomes of pediatric ocular myasthenia gravis
1Department of Ophthalmology, Vision Center at Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, Los Angeles, California, USA.
Insights
Pediatric ocular myasthenia gravis, primarily treated with pyridostigmine bromide, shows distinct outcomes in children under 12. Most cases stabilize, with a low rate of generalization to systemic disease.
Area of Science:
- Ophthalmology
- Neurology
- Pediatrics
Background:
- Ocular myasthenia gravis (OMG) is a rare autoimmune disorder affecting neuromuscular junctions in the eye.
- Understanding its presentation and progression in preadolescent children is crucial for effective management.
Purpose of the Study:
- To analyze the clinical characteristics, treatment strategies, and outcomes of purely ocular myasthenia gravis in children under 12 years old.
- To evaluate the efficacy of pyridostigmine bromide as a primary treatment for pediatric OMG.
Main Methods:
- Retrospective case series analysis of 21 patients under 12 years with initial presentation of purely ocular myasthenia gravis.
- Review of clinical charts to assess presenting signs, time to disease generalization or stabilization, and visual outcomes.
Main Results:
- The median age of onset was 26 months, with ptosis and strabismus being the most common presenting signs.
- Pyridostigmine bromide monotherapy was the initial treatment for all patients; 3 patients progressed to generalized myasthenia gravis.
- All patients achieved stabilization of ocular motor deficits, with complete resolution occurring in 4 patients; 2 patients had residual amblyopia.
Conclusions:
- Pediatric ocular myasthenia gravis presents differently from adult-onset disease, often before age 5.
- Pyridostigmine bromide is a safe and effective primary treatment, with a lower rate of generalization to systemic disease compared to adults.
- Ocular manifestations in children consistently stabilize, and some achieve complete resolution.
Purpose:
To review the presenting signs, therapeutic interventions, and clinical outcomes of purely ocular myasthenia gravis in a preadolescent population treated primarily with pyridostigmine bromide.
Design:
Retrospective case series.
Participants:
Twenty-one consecutive patients younger than 12 years with purely ocular myasthenia gravis at initial presentation.
Methods:
The clinical charts were reviewed retrospectively.
Main Outcome Measures:
Time to generalization, stabilization, or resolution; prism cover test results; and visual acuity.
Results:
Median age at onset was 26 months. The mean duration of follow-up was 6.5 years (range, 2-15). Presenting signs included ptosis (95%), strabismus (76%), limitation of ductions (17%), and Cogan's lid twitch (76%). The most common form of strabismus was exotropia. Pyridostigmine monotherapy was the initial treatment for all patients. Corticosteroids were added to the therapy for 6 patients. Generalization to systemic disease occurred in 3 patients. Two of those required intravenous immunoglobulin and thymectomy. Complete resolution off of medical therapy occurred in 4 patients. All patients had stabilization of the ocular motor deficits regardless of treatment. Eleven patients were treated for amblyopia; 2 had residual amblyopia.
Conclusions:
Ocular myasthenia in preadolescent children generally presents before age 5 and is clinically distinguished from the disease as it affects adults. Although the presenting signs of strabismus, ptosis, and Cogan's lid twitch are common in children or adults, the response to treatment and eventual outcomes differ. Most children can be safely treated with pyridostigmine alone. Generalization to systemic disease occurs at a much lower rate than in adults. Ocular manifestations stabilize in all children and completely resolve in some.
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