Jc. Rigalsastourne et al., OCULAR MANIFESTATIONS OF JUVENILE MYASTHE NIA-GRAVIS - OUTCOME AND MANAGEMENT, Annales de pediatrie, 43(5), 1996, pp. 342-347
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.