Gulati Sheffali, Jain Puneet, Kannan Lakshminarayanan, Sehgal Rachna, Chakrabarty Biswaroop
Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
Division of Pediatric Neurology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
J Child Neurol. 2015 Oct;30(11):1440-7. doi: 10.1177/0883073815569304. Epub 2015 Feb 23.
This study describes the clinical characteristics, treatment, and outcome of children with West syndrome in a tertiary care hospital in north India. Overall, 310 case records diagnosed from January 2009 to June 2012 were reviewed. The median age of onset of spasms was 5 months (interquartile range = 2.5-7 months). The predominant underlying etiology was perinatal cerebral ischemia (55%). Adrenocorticotropic hormone or oral steroids were received by 92% children, of whom 43% became seizure free. Median lag time for appropriate treatment initiation was significantly less in patients who became seizure free as compared to those with persisting seizures (11 vs 15 months, P = .001) soon after receiving treatment of choice. Later age at onset of spasms was associated with a favorable seizure outcome (P = .03). In a resource-limited setting, unawareness along with treatment costs and repeated visits to the hospital are significant obstacles to optimum management.
本研究描述了印度北部一家三级护理医院中患有韦斯特综合征儿童的临床特征、治疗及预后情况。总体而言,回顾了2009年1月至2012年6月期间诊断的310例病例记录。痉挛发作的中位年龄为5个月(四分位间距=2.5 - 7个月)。主要潜在病因是围产期脑缺血(55%)。92%的儿童接受了促肾上腺皮质激素或口服类固醇治疗,其中43%的儿童癫痫发作停止。与仍有癫痫发作的患者相比,癫痫发作停止的患者在接受首选治疗后,开始适当治疗的中位延迟时间显著更短(11个月对15个月,P = 0.001)。痉挛发作起始年龄较大与癫痫发作预后良好相关(P = 0.03)。在资源有限的情况下,缺乏认知以及治疗费用和多次前往医院就诊是实现最佳管理的重大障碍。