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Juvenile Myasthenia Gravis in North Texas: Clinical Features, Treatment Response, and Outcomes.
Lu, Marilyn; Reisch, Joan; Iannaccone, Susan; Batley, Kaitlin.
Afiliação
  • Lu M; Pediatric Neuromuscular Division, Departments of Pediatrics and Neurology, The University of Texas Southwestern Medical Center, Dallas, Texas. Electronic address: Marilyn.Lu@UTSouthwestern.edu.
  • Reisch J; Department of Population and Data Sciences, The University of Texas Southwestern Medical Center, Dallas, Texas.
  • Iannaccone S; Pediatric Neuromuscular Division, Departments of Pediatrics and Neurology, The University of Texas Southwestern Medical Center, Dallas, Texas.
  • Batley K; Pediatric Neuromuscular Division, Departments of Pediatrics and Neurology, The University of Texas Southwestern Medical Center, Dallas, Texas.
Pediatr Neurol ; 156: 10-14, 2024 Jul.
Article em En | MEDLINE | ID: mdl-38688232
ABSTRACT

BACKGROUND:

Juvenile myasthenia gravis (JMG) is a rare autoimmune disease that causes fatigable muscle weakness in children aged <18 years. There is currently no curative treatment or internationally accepted standard of care for JMG. The objective is to investigate relationships between clinical presentation, antibody status, severity of disease onset, electrodiagnostic evaluation, and response to therapy in JMG.

METHODS:

This study was a retrospective chart review. Congenital myasthenic syndromes were excluded. Data on demographics, treatments, and outcomes were collected. Disease severity was evaluated using Myasthenia Gravis Foundation of America (MGFA) clinical classifications.

RESULTS:

We identified 84 patients with JMG at Children's Medical Center Dallas between January 2014 and February 2022. It was found that 52% of patients presented with ocular JMG (median onset age 4.5 years) and 48% with generalized JMG (median onset age 11.5 years); 81% tested positive for acetylcholine receptor antibodies. Patients were 17% non-Hispanic white, 29% Hispanic, 39% black, and 12% Asian. There was a significant difference in average MGFA scores between ethnicities (P = 0.047) and age groups (P = 0.004), with postpubertal patients having higher average MGFA scores than prepubertal patients. Seventy-one percent of patients who underwent thymectomy experienced a decrease in MGFA scores postprocedure.

CONCLUSIONS:

Our study showed that there were significant differences in disease severity between ethnicities and age groups and that most patients who underwent thymectomy showed clinical improvement. These outcomes highlight the need for additional therapies in the treatment of JMG and the importance of extending clinical trials to the pediatric population.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Miastenia Gravis Limite: Adolescent / Child / Child, preschool / Female / Humans / Male País/Região como assunto: America do norte Idioma: En Revista: Pediatr Neurol Assunto da revista: NEUROLOGIA / PEDIATRIA Ano de publicação: 2024 Tipo de documento: Article País de publicação: Estados Unidos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Miastenia Gravis Limite: Adolescent / Child / Child, preschool / Female / Humans / Male País/Região como assunto: America do norte Idioma: En Revista: Pediatr Neurol Assunto da revista: NEUROLOGIA / PEDIATRIA Ano de publicação: 2024 Tipo de documento: Article País de publicação: Estados Unidos