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West's syndrome--etiology, treatment and prognosis
1Department of Paediatrics, Higher Medical Institute, Plovdiv, Bulgaria.
Insights
Synacthen effectively treats West's syndrome, especially when antiepileptic drugs fail. Lower doses are recommended to minimize severe side effects in children with this epilepsy type.
Area of Science:
- Pediatrics
- Neurology
- Epileptology
Background:
- West's syndrome is a severe infant epilepsy.
- Idiopathic cases are rare (9.5%), with most being symptomatic (90.5%).
- Perinatal factors are the leading cause (76.5%).
Purpose of the Study:
- To analyze the etiology, treatment outcomes, and developmental follow-up of children with West's syndrome.
- To evaluate the efficacy and safety of Synacthen in West's syndrome treatment.
- To compare different Synacthen dosages for optimal therapeutic benefit.
Main Methods:
- Retrospective analysis of 42 children with West's syndrome over 10 years.
- Follow-up duration ranged from 6 months to 10 years.
- Treatment involved antiepileptic drugs and Synacthen at varying doses (0.0125, 0.025, >=0.05 mg/kg/day).
Main Results:
- Complete seizure control was achieved in 40.5% with antiepileptic drugs alone.
- Synacthen treatment showed no statistically significant dose-dependent efficacy.
- Higher Synacthen doses correlated with more frequent and severe side effects.
- 88.1% of children experienced mental retardation and/or neurological deficits.
- One death occurred during Synacthen treatment; two more died within a year.
- One-third of cases transitioned to other epileptic syndromes.
Conclusions:
- Synacthen is efficacious for West's syndrome, particularly when initial treatments fail.
- Early introduction of Synacthen, preferably at lower doses, is advised to mitigate adverse effects.
- Long-term neurodevelopmental deficits remain a significant concern in treated children.
Abstract:
Forty two children with West's syndrome who had been treated in the Clinic of Paediatrics, Higher Medical Institute, Plovdiv in the last 10 years were entered into the present study. Analysis is made of the aetiology of the disease, the results of treatment and development of the children. All children were followed up from 6 months to 10 years. The West's syndrome was idiopathic in four children and symptomatic in 38 children (90.5%). It had perinatal aetiology in 76.5% of the patients, prenatal in 21%, and postnatal in 2.6%. Complete seizure control was achieved in 17 children (40.5%) treated only with antiepileptic drugs. Synacthen was included in the treatment of the remaining 22 children in three therapeutic doses--0.0125 mg/kg/day (n = 8), 0.025 mg/kg/day (n = 8), and > or = 0.05 mg/kg/day (n = 6). Treatment with different doses of Synacthen showed no statistically significant differences in the three groups. The side effects of the treatment occurred more frequently and were more severe in the groups with a high-dose Synacthen treatment. The follow-up established mental retardation and/or neurological deficit in 88.1% of the children. One infant died during the treatment with Synacthen and another two with severe mental retardation--one year after treatment. In about one third of the cases transition was observed to other epileptic syndromes. Synacthen is concluded to be efficacious in the treatment of West's syndrome. If antiepileptic drugs fail to produce any effect Synacthen should be included in the therapy in due time, preferably in small doses in order to avoid severe and unwanted side effects.