Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Short term outcomes of topiramate monotherapy as a first-line treatment in newly diagnosed West syndrome

  • Lee, Gyu-Min (Department of Pediatrics, Kyung Hee University School of Medicine) ;
  • Lee, Kyung-Suk (Department of Pediatrics, Kyung Hee University School of Medicine) ;
  • Lee, Eun-Hye (Department of Pediatrics, Kyung Hee University School of Medicine) ;
  • Chung, Sa-Jun (Department of Pediatrics, Kyung Hee University School of Medicine)
  • Received : 2011.06.22
  • Accepted : 2011.08.31
  • Published : 2011.09.15
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Abstract

Purpose: To investigate the efficacy of topiramate monotherapy in West syndrome prospectively. Methods: The study population included 28 patients (15 male and 13 female children aged 2 to 18 months) diagnosed with West syndrome. After a 2-week baseline period for documentation of the frequency of spasms, topiramate was initiated at 2 mg/kg/day. The dose was increased by 2 mg/kg every week to a maximum of 12 mg/kg/day. Clinical assessment was based on the parents' report and a neurological examination every 2 weeks for the first 2 months of treatment. The baseline electroencephalograms (EEGs) were compared with the post-treatment EEGs at 2 weeks and 1 month. Results: West syndrome was considered to be cryptogenic in 7 of the 28 patients and symptomatic in 21 patients. After treatment, 11 patients (39%) became spasm-free, 6 (21%) had more than 50% spasms-reduction, 3 (11%) showed less than 50% reduction, and 8 (29%) did not respond. The effective daily dose for achieving more than 50% reduction in spasm frequency, including becoming spasm-free, was found to be $5.8{\pm}1.1$ mg/kg/day. Nine patients (32%) showed complete disappearance of spasms and hypsarrhythmia, and 11 (39%) showed improved EEG results. Despite adverse events (4 instances of irritability, 3 of drowsiness, and 1 of decreased feeding), no patients discontinued the medication. Conclusion: Topiramate monotherapy seems to be effective and well tolerated as a first line therapy for West syndrome and is not associated with serious adverse effects.

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References

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