Treatment of preterm infants with West syndrome: differences due to etiology

Abstract

Background: This study was conducted with a particular focus on preterm infants with West syndrome (WS) to evaluate differences in the first responses to oral medication based on etiology.

Methods: Medical records of 53 patients with WS, treated at five institutions between 2005 and 2009, were reviewed retrospectively. Patients were divided into six groups based on the time of brain insult, and evaluated for short-term outcomes using oral anti-epileptic agents and synthetic adrenocorticotropic hormone.

Results: The sample consisted of 15, six, 14, two, four, and 12 patients classified, on the basis of apparent time of acquisition of etiology, into the prenatal, term, preterm, postnatal, other, and no identified etiology groups, respectively. Average age of onset in the term group was 3.3 ± 1.0 months, significantly earlier than in the prenatal, preterm, postnatal and no identified etiology groups (P < 0.05). All patients in the term group had experienced seizures before the onset of WS. Only patients in the preterm group had only experienced neonatal seizures, and responded better to treatment. Patients in the preterm group had better responses to treatment, especially oral medication, compared with those in the prenatal and term groups. The prevalence of relapse of seizures in the preterm group (14%) was significantly lower than that in the prenatal group.

Conclusions: Preterm WS patients responded well to treatment. Distinguishing WS patients on the basis of different etiologies is important for evaluating the effectiveness of treatment.