期刊
EPILEPSIA
卷 63, 期 2, 页码 352-363出版社
WILEY
DOI: 10.1111/epi.17125
关键词
CDKL5 deficiency disorder; developmental and epileptic encephalopathy; developmental trajectory; quality of life; seizure burden
资金
- Orphan Disease Center, University of Pennsylvania
- Australian National Health & Medical Research Council (NHMRC) [1117105]
- National Health and Medical Research Council of Australia [1117105] Funding Source: NHMRC
This study found that patients with better early seizure control showed slightly improved development later, suggesting that an individual child's trajectory is not necessarily predetermined and could possibly be influenced by optimal seizure management. This has implications for children's quality of life.
Objective The study investigated the effect of seizure and medication burden at initial contact with the International CDKL5 Disorder Database on subsequent development and clinical severity and compared quality of life among those whose development progressed, remained stable, or regressed between baseline and follow-up. Methods The effects of seizure and medication burden at baseline (high or low) on the CDKL5 Disorder Severity Scores and CDKL5 Developmental Score (CDS) at follow-up were assessed using linear and negative binomial regressions, respectively, with adjustment for age at baseline, gender, and follow-up duration with and without genotype. Seizure and medication burden were defined by average daily seizure count (high, >= 5/day; low, <5/day) and number of antiseizure medications (high, >= 3/day; low, <3/day), respectively. The effects of change in CDS over time (improved, stable, or deteriorated) on Quality of Life Inventory-Disability (QI-Disability) total and domain scores at follow-up were assessed in those aged at least 3 years at follow-up using linear regression models with adjustment for baseline CDS, gender, and follow-up duration. Results The expected follow-up CDS was lower for individuals with high compared to low seizure burden at baseline (beta = -.49, 95% confidence interval [CI] = -.84 to -.13). The average total QI-Disability score was 5.6 (95% CI = -.2 to 11.5) points higher among those with improved compared with stable or deteriorating CDS and 8.5 (95% CI = 3.1-13.8) points lower for those with deteriorating compared to stable or improved CDS. Significance Our finding that later development showed slight improvement in those with better earlier seizure control even after adjustment for genotype suggests that the trajectory for an individual child is not necessarily predetermined and could possibly be influenced by optimal seizure management. This has implications for children's quality of life.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据