Gupta Juhi, Sharma Suvasini, Mukherjee Sharmila B, Jain Puneet, Aneja Satinder
Department of Pediatrics, Lady Harding Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India.
Department of Pediatric Neurology, Lady Harding Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India.
Ann Indian Acad Neurol. 2020 Mar-Apr;23(2):177-181. doi: 10.4103/aian.AIAN_503_18. Epub 2020 Feb 25.
To assess the neurodevelopmental outcome of West syndrome (WS) in Indian children, who differ in their clinical profile from the western population.
This cross-sectional study enrolled children aged 2--5 years with prior diagnosis of WS between November 2013 and March 2015. They were assessed for epilepsy outcome and developmental outcome using developmental profile 3 (DP3) and vineland adaptive behavioral scale II (VABS II).
Sixty-one children were enrolled. Perinatal asphyxia (40.9%), neonatal hypoglycemia (14.8%), and neonatal meningitis (9.8%) were predominant causes among the children with known etiology. Favorable epilepsy outcome (seizure freedom for >6 months) was observed in 29/61 patients (47.5%). Moderate to severe developmental delay was observed in 55/61 children (91.8%). Favorable developmental outcome (GDS by DP3 >70) was observed in just 5/61 (8%) patients.
This study highlights the high prevalence of developmental delay in this population of children with WS, with adverse perinatal events being the most common etiology.
评估印度儿童韦斯特综合征(WS)的神经发育结局,这些儿童的临床特征与西方人群不同。
这项横断面研究纳入了2013年11月至2015年3月期间先前被诊断为WS的2至5岁儿童。使用发育量表3(DP3)和文兰适应行为量表II(VABS II)对他们进行癫痫结局和发育结局评估。
共纳入61名儿童。在病因已知的儿童中,围产期窒息(40.9%)、新生儿低血糖(14.8%)和新生儿脑膜炎(9.8%)是主要病因。29/61例患者(47.5%)观察到良好的癫痫结局(无癫痫发作>6个月)。55/61例儿童(91.8%)观察到中度至重度发育迟缓。仅5/61例(8%)患者观察到良好的发育结局(DP3的GDS>70)。
本研究强调了这群WS儿童中发育迟缓的高患病率,围产期不良事件是最常见的病因。