Early-onset myasthenia gravis: Clinical characteristics and response to therapy

Abstract

We studied 59 children with myasthenia gravis (MG). Disease onset was pre-pubertal in 26 patients and post-pubertal in 33. The male to female ratio was 0.62 in the early- and 0.17 in the late-onset groups. The frequency of ocular MG was higher in patients with prepubertal onset. Patients with generalized MG generally showed a good response to thymectomy and corticosteroid therapy proved effective with no major side-effects. In our experience early-onset MG has the same course as in adult life. We recommend thymectomy for generalized disease in childhood, except in very young children on account of possible long-term effects on immunological development. Immunosuppressive therapy should be considered in severely affected patients who do not respond adequately to other therapies.

Juvenile myasthenia gravis

“…The incidence of juvenile patients who present with purely ocular symptoms (Osserman grade Ia) has been reported to range widely from 27% to 93% in retrospective analyses (Table 1). 4,5,13,17,22,28,29,36,41,43 The variability in presentation appears to be somewhat dependent upon ethnicity. Several studies of Asian children have reported a substantially higher incidence of ocular presentation.…”

“…An analysis of 13 studies that cited the gender distribution of JMG patients revealed a female predominance of between 50% and 86%. [3][4][5]13,22,28,29,36,37,41,43,44 However, prepubertal-age patients (Ͼ12 years of age) ranged from 53% to 73% female, whereas postpubertal-age patients ranged from 77% to 85% female. 3,5,13,28,37 One recent study indicated that patients diagnosed before 10 years of age were equally distributed between male and female, but after age 10 females were five times as likely (P Ͻ 0.01) to be diagnosed in a ratio similar to that for adults.…”

“…Both studies included Ͼ50 patients and reported ocular-only presentation rates of between 26% and 31% for prepubescent patients, but rates of 9 -16% for pubescent and postpubescent patients. 5,13 A range of 29 -75% of JMG patients have been reported to have generalized disease, either at presentation or later. These figures are below the 85% estimated frequency in adults.…”

Myasthenia gravis in children: analysis of 18 patients

“…We used neostigmine that showed a positivity similar to the traditional Tensilon test 2,15,16 . We found the longer action of neostigmine somewhat advantageous since we had more time to observe the positive signs, especially in the very young.…”

Neuromuscular junctional disorders

“…2 Indian study of 77 children with MG has revealed an equal sex ratio. 3 MG is caused by serum antibodies(produced mainly by B cells) binding to the nicotine AChR on the post synaptic surface of the NMJ leading to its loss.…”