David Tvildiani Medical University, Tbilisi, Georgia.
David Tvildiani Medical University, Tbilisi, Georgia, M. Iashvili Children's Central Hospital - Neuroscience, Tbilisi, Georgia.
Epileptic Disord. 2022 Apr 1;24(2):359-372. doi: 10.1684/epd.2021.1397.
Infantile spasms syndrome is a severe epileptic encephalopathy. Management of infantile spasms remains challenging because of pharmacoresistant forms and relapsing seizures. A high number of patients with this syndrome have neurodevelopmental delay. The main objective of our study was to determine predictors to measure the neurodevelopmental outcome of patients with infantile spasms.
We prospectively evaluated 31 patients with infantile spasms from 2014 to 2017 at three hospitals in Tbilisi, Georgia. Various demographic data were evaluated at the first visit; video-EEG, brain MRI and neurodevelopmental evaluation were performed upon admission. A diary to record spasms was provided and completed by all parents/caregivers. Seizures were recorded on video and the phenomenology of infantile spasms was studied. Children were followed for one and two years after the first assessment.
Neurodevelopmental deterioration was revealed in 61.1% on the second and 53% on the third evaluation in patients with onset of spasms before seven months of age. The mean score on the ASQ communication domain was low among structural cases. Eleven patients with pre-existing delay had developmental regression based on the second evaluation (Fisher's exact test: 7.2; df 1; p=0.01).
Our study reveals that age at onset of infantile spasms at less than seven months, pre-existing developmental delay, low ASQ scores and structural abnormalities on MRI are predictors of poor developmental outcome. Our data suggest that clinicians should inform parents at the first clinical evaluation about prognosis, and intervention should be started as early as possible in order to improve development.
婴儿痉挛综合征是一种严重的癫痫性脑病。由于耐药形式和反复发作的癫痫，婴儿痉挛的治疗仍然具有挑战性。该综合征的许多患者存在神经发育迟缓。我们研究的主要目的是确定预测指标，以衡量婴儿痉挛患者的神经发育结局。
我们前瞻性评估了 2014 年至 2017 年格鲁吉亚第比利斯的三家医院的 31 例婴儿痉挛患者。在首次就诊时评估了各种人口统计学数据；入院时进行了视频脑电图、脑 MRI 和神经发育评估。提供了一份记录痉挛的日记，并由所有父母/照顾者填写。记录视频中的癫痫发作，并研究婴儿痉挛的现象学。在首次评估后的一到两年内对儿童进行随访。
在发病年龄小于七个月的患者中，第二次和第三次评估分别有 61.1%和 53%显示神经发育恶化。在结构性病例中，ASQ 沟通领域的平均得分较低。11 名存在发育迟缓的患者根据第二次评估出现发育倒退（Fisher 确切检验：7.2；df 1；p=0.01）。
我们的研究表明，发病年龄小于七个月、存在发育迟缓、ASQ 评分低和 MRI 上存在结构性异常是发育不良结局的预测因素。我们的数据表明，临床医生应在首次临床评估时告知家长预后，并应尽早开始干预，以改善发育。
