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Published on: March 22, 2017
[Proposals for the treatment of children with West's syndrome]
1Servicio de Neuropediatría, Hospital Universitario Marquís de Valdecilla, Santander, Spain. jherranz@nacom.es
Insights
Treatment guidelines for infantile spasms (West's syndrome) recommend starting with prednisone for idiopathic cases and vigabatrin for symptomatic cases. Adjustments to vigabatrin, prednisone, topiramate, or valproate are suggested based on efficacy and side effects.
Area of Science:
- Pediatric Neurology
- Clinical Pharmacology
Context:
- Infantile spasms, also known as West's syndrome, represent a severe epilepsy syndrome in infants.
- Establishing effective treatment protocols is crucial for improving outcomes and reducing long-term neurological deficits.
Purpose:
- To review and justify treatment guidelines for infantile spasms (West's syndrome) in children.
- To evaluate the efficacy and side-effects of various pharmacological interventions based on prospective studies.
Summary:
- For idiopathic or cryptogenic West's syndrome, initial treatment with oral prednisone (up to 8 mg/kg/day) is recommended, transitioning to vigabatrin if ineffective or poorly tolerated.
- For secondary or symptomatic West's syndrome, vigabatrin (up to 200 mg/kg/day) is the preferred first-line treatment, with prednisone as an alternative if vigabatrin fails or causes side effects.
- If initial treatments are unsuccessful, topiramate (up to 24 mg/kg/day) or valproate (up to 200 mg/kg/day) are suggested as subsequent therapeutic options.
Impact:
- Provides evidence-based recommendations for the management of West's syndrome, aiming to optimize treatment strategies.
- Guides clinicians in selecting appropriate first-line and alternative therapies based on the etiology and response of infantile spasms.
Objective:
The justification of guidelines for the treatment of children with infantile spasms and West's syndrome.
Development:
We review, by means of prospective studies of suitable methodology, the efficacy and side-effects of adrenocorticotropin, sodium valproate, vigabatrin and other drugs used in the treatment of children with West's syndrome.
Conclusions:
In view of the information obtained from these studies, we suggest that in children with idiopathic or cryptogenic West's syndrome, treatment should be started with oral prednisone up to a dose of 8 mg/kg/day which should be changed to vigabatrin in the case of inefficacy or side effects. In children with secondary or symptomatic West's syndrome, treatment should be started with vigabatrin, up to a maximum dose of 200 mg/kg/day and if this is ineffective or there are side-effects it should be replaced by prednisone. If neither of these forms of treatment are successful, topiramate (up to 24 mg/kg/day) or valproate (up to 200 mg/kg/day) should be given.
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