Long-term Outcomes of Pediatric Ocular Myasthenia Gravis
Received 5 June 2007; received in revised form 13 October 2007; accepted 16 October 2007. published online 03 January 2008.
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.
Available online: December 26, 2007.
Department of Ophthalmology, Vision Center at Childrens Hospital Los Angeles, University of Southern California/Keck School of Medicine, Los Angeles, California.
Manuscript no. 2007-754.
The authors have no financial interest in any product mentioned in the article.
Correspondence and reprint requests to Childrens Hospital Los Angeles M.S. 88, 4650 Sunset Boulevard, Los Angeles, CA 90027.
ABSTRACT