Department of Pediatrics Neurology and Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran.
Department of Pediatrics Neurology and Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran.
Res Dev Disabil. 2019 Jun;89:114-119. doi: 10.1016/j.ridd.2019.03.010. Epub 2019 Apr 8.
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.
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).
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.
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.
本研究旨在通过使用婴儿发育贝利三世量表，作为来自中东的首次报告，调查 West 综合征 (WS) 患儿的临床特征和神经发育结局。
在 2013 年 1 月至 2016 年 2 月期间，我们前瞻性地招募了来自伊朗伊斯法罕的 67 例确诊为 WS 的连续患者。使用贝利婴幼儿发育量表第三版（贝利三世）评估研究对象的认知、语言和运动结局。
总体而言，研究共纳入 67 例病例，包括 34 例（50.7%）男孩和 33 例（49.3%）女孩（男女比例为 1.03）。平均年龄为 26.7±12.9 个月。在这些患者中，50 例（74.6%）患者为症状性 WS，17 例（25.4%）患者为隐源性 WS。认知评估中 76.9%的患者存在“严重延迟”，67.7%的患者存在语言和交流能力延迟，81.5%的患者存在运动功能延迟。与有症状性 WS 的患者相比，隐源性 WS 的患者在运动（p=0.035）、认知（p=0.035）和接受性语言（p=0.043）方面的结果明显更好。与未控制发作的患者相比，控制发作的患者在运动（p=0.027）和认知（p=0.011）方面的结果明显更好。
在我们的研究中，WS 与神经发育不良结局相关。严重的发育迟缓与两个主要因素有关：（i）存在特定的潜在异常（症状性 WS）和（ii）由于前者导致的持续性发作。
