소아기 중증근무력증 27례에 대한 임상적 고찰

A Clinical Study on Myasthenia Gravis in Childhood

Twenty-seven cases of myasthenia gravis in childhood were diagnosed and treated in the Department of Pediatrics, Inje University Hospital from February 1981 till February 1994 were reviewed clinically. Mean age of myasthenia gravis was 5.02±3.11 years of age and 16 cases(59.3%) were below the age of 5 years. Male to female ratio was 1:1.7. Most common initial clinical manifestation was ptosis noted all cases, followed by strabismus, diplopia, and impairment of lateral gaze. Edrophonium and neostigmine test were positive in all cases and antiacetylcholine receptor antibody was posiive in two cases among 3 in which antiacetylcholine receptor antibody study was done. There was no associated disease except one case of hyperthyroidism. Anticholinesterase therapy was tried in all case with 11 cases of remission, and predisolone was added in 12 cases who were not respond to anticholinesterase therapy with 9 cases of remission. Response to mestinon was not so effective(40.7%) and steroid was effective in juvenile myasthenia gravis that didn't respond to anticholinesterase therapy.