Document Detail


Long-term outcomes of pediatric ocular myasthenia gravis.
MedLine Citation:
PMID:  18155768     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Steve Ortiz; Mark Borchert
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Publication Detail:
Type:  Journal Article     Date:  2007-12-26
Journal Detail:
Title:  Ophthalmology     Volume:  115     ISSN:  1549-4713     ISO Abbreviation:  Ophthalmology     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-07-07     Completed Date:  2008-07-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7802443     Medline TA:  Ophthalmology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1245-1248.e1     Citation Subset:  IM    
Affiliation:
Department of Ophthalmology, Vision Center at Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, Los Angeles, California, USA.
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MeSH Terms
Descriptor/Qualifier:
Blepharoptosis / diagnosis*
Child
Child, Preschool
Cholinesterase Inhibitors / therapeutic use
Drug Therapy, Combination
Female
Follow-Up Studies
Glucocorticoids / therapeutic use
Humans
Immunoglobulins, Intravenous / therapeutic use
Infant
Male
Myasthenia Gravis / diagnosis*,  drug therapy
Ocular Motility Disorders / diagnosis*
Pyridostigmine Bromide / therapeutic use
Retrospective Studies
Strabismus / diagnosis*
Thymectomy
Treatment Outcome
Visual Acuity
Chemical
Reg. No./Substance:
0/Cholinesterase Inhibitors; 0/Glucocorticoids; 0/Immunoglobulins, Intravenous; 101-26-8/Pyridostigmine Bromide

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