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[West's syndrome. A clinical, therapeutic and prognostic study. Apropos of 66 cases]
Insights
Early treatment for West syndrome, particularly in cryptogenic forms and infants aged 6-8 months, yields the best outcomes. Various therapies showed no significant differences, emphasizing timely intervention for infantile spasms.
Area of Science:
- Pediatrics
- Neurology
- Clinical Medicine
Background:
- West syndrome, a severe form of epilepsy, affects infants.
- Characterized by infantile spasms, developmental regression, and hypsarrhythmia.
- Long-term outcomes and effective treatments remain critical research areas.
Purpose of the Study:
- To review the long-term outcomes of West syndrome.
- To evaluate the effectiveness of different therapeutic interventions.
- To identify factors influencing treatment success in West syndrome.
Main Methods:
- Retrospective chart review of 66 children with West syndrome.
- Data collected from 1957 to 1984 at University Central Hospital, Lille.
- Analysis of disease onset, etiology, clinical features, and treatment responses.
Main Results:
- Most common age at onset was 3-5 months (37.8%) and 6-8 months (30.3%).
- Prenatal causes were significant (42.4%), with unknown etiology in 27.3%.
- Early treatment initiation in cryptogenic forms and older infants (6-8 months) showed the best results, irrespective of therapy type.
Conclusions:
- Timely intervention is crucial for improving West syndrome outcomes.
- Cryptogenic West syndrome and onset between 6-8 months may benefit most from early treatment.
- Further research into specific etiological factors and tailored therapies is warranted.
Abstract:
The charts of 66 children suffering from West syndrome followed by the paediatric department of the University Central Hospital, Lille, between 1957 and 1984 were reviewed: 24 cases were followed for more than 10 years; 20 cases from 5 to 10 years; 22 cases from 1 to 5 years. Age at onset of the disease ranged from 3 to 5 months (37.8%) and 6 to 8 months (30.3%) with a slight male predominance. Prenatal causes were the most important (42.4%) with anomalies and cerebral malformations in 15.2% and neurocutaneous syndromes in 9.1% of cases. Etiology remained unknown in 27.3% of cases. Some clinical aspects were characteristic (flexion or extension spasms, psychomotor retardation) in 84.8% of cases. Hyparrhythmia occurred in 62.1% of cases. There were no significant differences in the results of the varying therapies used (ACTH, Synacten, hydrocortisone) or its associations. The best results were obtained with a treatment started as early as possible in the cryptogenic forms and in older infants (6 to 8 months).