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[Myasthenia in children: long-term course]
H Descamps1, J Bataille, B Estournet
1Service de Réanimation Infantile, Rééducation Neuro-Respiratoire, Hôpital Raymond-Poincaré, Garches.
Insights
For pediatric myasthenia gravis, thymectomy and steroid therapy offer limited benefits and potential dependence. Researchers suggest avoiding routine thymectomy and minimizing steroid use in children with myasthenia gravis.
Area of Science:
- Neurology
- Pediatrics
- Immunology
Context:
- Pediatric myasthenia gravis presents unique challenges in diagnosis and treatment.
- Respiratory complications are common, affecting over 60% of pediatric patients.
- Familial and pure ophthalmic forms were not observed in this cohort.
Purpose:
- To evaluate the long-term efficacy and safety of thymectomy and steroid therapy in pediatric myasthenia gravis.
- To determine optimal treatment strategies for children diagnosed with myasthenia gravis.
- To assess the impact of surgical intervention versus conservative management.
Summary:
- A 10-year follow-up of 23 children with myasthenia gravis revealed limited efficacy of thymectomy, with symptom stability or worsening in 75% post-surgery.
- Steroid therapy showed mixed results: ineffective short courses in younger children and cortico-dependence in 4 of 6 older children.
- Complete remission rates were similar between thymectomized and non-thymectomized groups at 7 years post-surgery.
Impact:
- Findings challenge the routine use of thymectomy for pediatric myasthenia gravis.
- Suggests a conservative approach, minimizing steroid therapy due to potential adverse effects like cortico-dependence.
- Highlights the need for individualized treatment plans based on patient age and disease severity.
Abstract:
Twenty three children with myasthenia were followed up in our Department for 10 years. There were no familial forms of the disease; there were no patients with the pure ophthalmic form but 61 p. 100 of patients had serious respiratory disease. Steroid therapy was possible in 10 cases: in 4 patients under 15 years of age, short courses of steroids were ineffective; in 6 patients over the age of 15, long term steroid therapy resulted in some improvement but cortico-dependence developed in 4 cases. One child responded to treatment with veinoglobulin (R). Sixteen children underwent thymectomy; the symptoms in the 7 non-thymectomized patients were equivalent. The myasthenic syndrome was stable or worsened in 75 p. 100 of cases after thymectomy; only 56 p. 100 of children improved 2 years after surgery. The incidence of complete remission was the same in the children undergoing thymectomy and in those not treated surgically at 7 years after thymectomy. Our attitude is therefore not to refer myasthenic children for routine thymectomy and to avoid steroid therapy whenever possible.