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.
Highlights
Myasthenia gravis (MG) is an autoimmune disease primarily mediated by the anti-acetylcholine receptor antibody (AchR‐Ab), which is associated with a variety of immune molecules and occurs in neuromuscular junctions [1,2]
The pathophysiology involves the production of AchR-Ab and the inhibition of AchR, reducing the number of functional AchR in the neuromuscular junction, which leads to an abnormal decline in the motor endplate membrane potential
Studies conducted in Europe and the United States have reported a prevalence of 14‐1.5/1.0x105, with an uncommon MG onset during childhood (10‐15%) [10]
Summary
Myasthenia gravis (MG) is an autoimmune disease primarily mediated by the anti-acetylcholine receptor antibody (AchR‐Ab), which is associated with a variety of immune molecules and occurs in neuromuscular junctions [1,2]. We present the conclusions drawn from the age-related clinical characteristics, treatment and prognosis
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