Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
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Published on: October 25, 2018
Zhi-Xiao Yang1, Kai-Li Xu1, Hui Xiong2
1Department of Neurology, Children's Hospital of Zhengzhou, Zhengzhou, Henan 450053, P.R. China.
Insights
Childhood myasthenia gravis (MG) is often ocular type, with low rates of systemic progression or myasthenia crisis. Steroid treatment proved effective for most pediatric MG patients.
Area of Science:
- Pediatric Neurology
- Immunology
- Clinical Medicine
Background:
- Childhood myasthenia gravis (MG) presents unique diagnostic and therapeutic challenges.
- Understanding the clinical spectrum and prognostic factors is crucial for effective management.
Purpose of the Study:
- To analyze the clinical characteristics, classification, and treatment of pediatric myasthenia gravis.
- To evaluate the prognosis of childhood MG through follow-up assessments.
Main Methods:
- Retrospective analysis and prospective monitoring of clinical data from 135 children with MG.
- Grouping patients by clinical type and therapeutic agents.
- Assessing antibody status, electrophysiological tests, and immunological markers.
Main Results:
- 85.2% of patients presented with ocular type (Type I) MG, with a low progression rate to systemic forms.
- Relapse occurred in 46.1% of followed patients; acetylcholine receptor antibody positivity was 40.19%.
- Decreased CD4+, CD8+, or CD3+ expression was noted in 71% of patients; thymic hyperplasia and thymoma were rare.
Conclusions:
- Ocular type MG is prevalent in children, with generally favorable outcomes and low incidence of myasthenia crisis.
- Steroid therapy is effective for the majority of pediatric MG cases.
- Limited neurological sequelae and rare thymoma suggest a generally good prognosis for childhood MG.
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.
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