West syndrome: response to valproate.

Area of Science:

• Pediatric Neurology
• Epileptology
• Clinical Pharmacology

Background:

• West syndrome, characterized by infantile spasms, presents management challenges.
• Current treatment outcomes for West syndrome are often unsatisfactory.
• Valproate is a commonly used antiepileptic drug with potential utility in West syndrome.

Purpose of the Study:

• To prospectively evaluate the effectiveness of valproate in controlling infantile spasms in children diagnosed with West syndrome.
• To identify predictors of response and relapse to valproate treatment.

Main Methods:

• A prospective study enrolled consecutive West syndrome patients.
• Valproate was administered at 30 mg/kg/day to treatment-naive patients.
• Patients were monitored bi-weekly, with hormonal therapy used for partial/poor responders or relapsed cases.

Main Results:

• Of 91 patients initiated on valproate, 36 (39.5%) achieved a good response, though seven relapsed.
• Later age at onset and typical hypsarrhythmia predicted sustained response; delayed cry at birth predicted poor response.
• Hormonal therapy in 62 patients with poor response or relapse yielded a 58.1% good response rate, with some relapses after discontinuation.

Conclusions:

• Valproate demonstrates efficacy in a subset of West syndrome patients.
• Patient characteristics, such as age at onset and EEG findings, can guide treatment selection.
• Further research may refine the role of valproate and hormonal therapy in West syndrome management.