OCULAR MANIFESTATIONS OF JUVENILE MYASTHE NIA-GRAVIS - OUTCOME AND MANAGEMENT

Citation
Jc. Rigalsastourne et al., OCULAR MANIFESTATIONS OF JUVENILE MYASTHE NIA-GRAVIS - OUTCOME AND MANAGEMENT, Annales de pediatrie, 43(5), 1996, pp. 342-347
Citations number
17
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
00662097
Volume
43
Issue
5
Year of publication
1996
Pages
342 - 347
Database
ISI
SICI code
0066-2097(1996)43:5<342:OMOJMN>2.0.ZU;2-H
Abstract
Juvenile myasthenia gravis is an uncommon autoimmune disease character ized by production of antibodies to acetylcholine receptors in the neu romuscular junction. Four cases of myasthenia gravis with isolated ocu lar manifestations in children aged two to ten years are reported. Pto sis was a consistent symptom and was the most common manifestation of extraocular muscle weakness. The variability of ptosis over time is ty pical of the condition. The edrophonium chloride test and assays of an tibodies to acetylcholine receptors are the most reliable diagnostic i nvestigations. Anticholinesterase drugs should be used as first-line t herapy for ocular manifestations of myasthenia gravis, Intravenous imm unoglobulins and/or thymectomy may be indicated in some cases. Periodi c ophthalmological evaluations should be done even in patients whose d isease is well controlled because the oculomotor disturbances can resu lt in loss of vision in the affected eye, of which the child or family may not be aware.