Zonisamide in West syndrome: an open label study

Area of Science:

• Pediatric Neurology
• Epileptology

Background:

• Infantile spasms (IS) are a severe epilepsy syndrome in infants.
• Conventional treatments like adrenocorticotropic hormones and vigabatrin have limitations.
• Novel therapeutic strategies for IS are needed.

Purpose of the Study:

• To evaluate the efficacy and tolerability of high-dose zonisamide in infants with recent-onset infantile spasms.
• To determine the optimal dosage and response rates of zonisamide in this population.

Main Methods:

• Seventeen infants with newly diagnosed infantile spasms were enrolled.
• Zonisamide was administered at escalating doses (2-30 mg/kg/day).
• Complete response was defined as 28 days of seizure cessation and hypsarrhythmia resolution on EEG.

Main Results:

• Overall, 42% of cryptogenic and 40% of symptomatic patients achieved complete response.
• Effective zonisamide doses ranged from 10-22 mg/kg/day.
• Adverse effects included irritability and poor oral intake; seizure recurrence was noted in 3/7 responders.

Conclusions:

• High-dose zonisamide appears to be an effective and safe option for some infants with newly diagnosed West syndrome.
• Further research is warranted to confirm these findings and optimize treatment protocols.