AUTHOR=Lin Yangtao , Kuang Qianjin , Li Hongjin , Liang Bo , Lu Jiaxin , Jiang Qilong , Yang Xiaojun 

TITLE=Outcome and clinical features in juvenile myasthenia gravis: A systematic review and meta-analysis

JOURNAL=Frontiers in Neurology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1119294

DOI=10.3389/fneur.2023.1119294

ISSN=1664-2295

ABSTRACT=Background: Juvenile myasthenia gravis (JMG) is a rare autoimmune disease and is only described in small cohort studies. We define clinical features, treatment, and outcomes of patients with JMG over the last 22 years.
Methods: A search of PubMed, EMBASE, and web of science (1/2000–2/2022) identified all English language and human studies of JMG. The population was patients diagnosed with JMG. Outcomes included the history of myasthenic crisis, autoimmune comorbidity, mortality, and treatment outcome. Data extraction was performed by independent reviewers. And we performed a pooled reanalysis of all published data in the included studies and compared with other studies of adult cohorts.
Results: We identified 12 articles describing 1223 patients diagnosed between 2006 and 2021. JMG occurred in 60.7% of female patients. The mean age at presentation was 7.38 years and ocular symptoms were the first clinical manifestation of JMG in 63.7%. The most common initial presentation was ptosis, occurring in 77.7%. AchR-Ab showed positive in 78.6%. 641 patients received thymus examination, found to have thymic hyperplasia in 59.0% and thymoma in 2.6%. Autoimmune comorbidity was found in 13.6% and the most common one is thyroid disease (61.5%). First-line therapy, consisting of pyridostigmine and steroids, was initiated in 99.5% and 70.4%, respectively. Spontaneous remission patients were achieved in 6 patients without any treatment. Thymectomy was performed in 41.8%. 9.7% of patients had a history of myasthenic crisis. Completely stable remission was achieved in 23.7% and mortality was reported in only 1 study, which reported 2 deaths.
Conclusion: JMG is a rare disease with a relatively benign course, and differs from adult MG in some clinical features. The treatment regimen guideline for children is still not well-established. There is a need for prospective studies to properly evaluate treatment regimes.