Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Abstract

This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4+, CD8+, or CD3+ was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae.

Clinical characteristics and outcome predictors of a Chinese childhood-onset myasthenia gravis cohort

“…Consistent with previous studies ( 6 , 11 ), we found that 96.2% of the patients presented with ocular symptoms only at onset, ptosis was the most common symptom, and 86.6% remained with OMG at the end of point when we followed up 164 cases for longer than 2 years. Similar to previous study ( 13 ), thyroid-related abnormalities and family history of autoimmune diseases were common in our study, indicating thyroid function as well as the related autoantibodies should be examined routinely for CMG patients.…”

“…Previous studies from China reported an AChR-Abs positivity rate between 40% and 78% ( 6 , 13 ), while this rate was relatively higher in other countries ( 8 , 14 ). In our current study, the positivity rate of AChR-Abs was 42.5%.…”

“…In this study, we have described clinical characteristics of our 343 CMG patients, it is one of the biggest CMG cohorts reported yet. Previous studies in China showed that CMG manifested mainly before puberty, the most common onset symptom was ptosis, and the rate of generalization of CMG was pretty low (less than 20%) ( 6 , 11 , 13 ). In our study the median onset age was 34 months with a peak between 1 and 7 years (84.2% in total), the male-to-female ratio was 1:1.5 showing a minor female predominance.…”

Juvenile Ocular Myasthenia Gravis: A Case Report

“…Corticosteroid, steroid sparing agents like azathrioprine, cyclosporine A, cyclophosphamide even plasma exchange / IVIG administration had been tried in some studies and showed promising results. Prednisone and prednisolone are the first-line therapy in children with persisting symptoms with the starting dosage is 0.5-1mg/kg (maximum 30 mg/day), with a possible increase up to 2 mg/kg/day (maximum 60-80mg/day) [31].…”

Pediatric Ocular Myasthenia Gravis: A Review

“…[10][11][12] In ►Table 1, demographic profile of children with JMG (both generalized and ocular) has been shown as per studies in different populations. 7,[13][14][15][16] These epidemiological differences suggest the effect of both genetic and environmental factors on type and severity of myasthenia, which might affect therapeutic aspects and prognosis of this disorder.…”