Neurodevelopmental outcomes of the West syndrome in pediatric patients: The first report from the Middle-East

Abstract

Background: This study aimed to investigate the clinical characteristics and neurodevelopmental outcomes of children with West syndrome (WS) by using the Bayley-III scale of infant development, as the first report from the Middle-East. Methods: Between January 2013 and February 2016, we prospectively enrolled 67 consecutive patients with a confirmed diagnosis of WS from Isfahan, Iran. Cognition, language and motor outcomes of the studied subjects were evaluated with the Bayley Scales of Infant and Toddler Development-Third Edition (Bayley-III). Results: Overall, 67 cases, including 34 (50.7) boys and 33 (49.3) girls (a male/female ratio of 1.03), were enrolled for the study. The mean age was 26.7 +/- 12.9 months. Among the subjects, 50 (74.6) patients had symptomatic WS, and 17 (25.4) patients had cryptogenic WS. "Severe delay" was found in 76.9 of the patients regarding cognitive evaluation, 67.7 for language and communication abilities, and, 81.5 for motor function. The patients with cryptogenic WS were significantly more likely to have more favorable outcomes in motor (p = 0.035), cognitive (p = 0.035) and receptive language (p = 0.043) in comparison to those who had symptomatic WS. The patients with controlled seizures were significantly more likely to have more favorable outcomes in motor (p = 0.027) and cognition (p = 0.011) as compared to those with uncontrolled seizures. Conclusion: WS was associated with poor neurodevelopmental outcome in our study. Severe developmental delay was associated with two major factors: (i) presence of a specific underlying abnormality (symptomatic WS) and(ii) persistent seizures as a result of the former.