Infantile myasthenia remains ocular and usually evolves favourably

A retrospective study of 859 patients followed by a Shanghai hospital for Myasthenia gravis diagnosed before the age of 14 found :

  • an ocular form in 97.8% of cases, which only generalized in 14% of patients;
  • 21.7% were in stable complete remission, 15.3% in pharmaceutical remission, and 42% of patients had achieved minimal manifestation status by the time of their last consultation, five to 16 years after the onset of the disease;
  • of the 2.1% of patients who had at least one myasthenic attack, none died;
  • the onset of myasthenia before puberty is predictive of a favorable outcome.

A second multicenter study conducted in the United States on 451 children with myasthenia, 238 of them treated with thymectomy, confirmed the efficacy of this procedure, with :

  • from one year before thymectomy to three years after,
  • a reduction in drug treatment, myasthenia-related hospitalizations and associated costs.

It should be noted that the surgical approach does not appear to influence the outcome of thymectomy.

 

Clinical Features, Treatment, and Prognostic Factors of Childhood-Onset Myasthenia Gravis in a Large Chinese Cohort. Bi Z, Ge H, Zhang Q et al. Pediatr Neurol. 2023 Sep;146:31-39

 

Measuring the Efficacy of Thymectomy for Pediatric Myasthenia Gravis Across Tertiary Children’s. De Boer C, Zeineddin S, Ott K et al. Hospitals. Pediatr Neurol. 2023 Aug 5;148:17-22