Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, Massachusetts.
Maine Medical Partners Group, Scarborough, Maine.
Epilepsia. 2020 Apr;61(4):702-713. doi: 10.1111/epi.16468. Epub 2020 Mar 5.
To determine risk factors and causes for mortality during childhood in patients with infantile spasms (IS). We describe the overall goals of care for those who died.
This is a retrospective chart review of IS patients born between 2000 and 2011. We examined potential risk factors for mortality, including etiology, neurologic impairment, medication use, persistence of epileptic spasms, and comorbid systemic involvement (requirement for G-tube feedings, respiratory interventions). For patients who died, we describe cause of death and resuscitation status or end-of-life care measures.
We identified 150 IS patients with median follow-up of 12 years. During the study period, 25 (17%) patients died, 13 before 5 years of age. Univariate analysis demonstrated that developmental delay, identifiable etiology, hormonal use for IS, persistence of epileptic spasms, polypharmacy with antiseizure medications, refractory epilepsy, respiratory system comorbidity, and the need for a G-tube were significant risk factors for mortality. In a multivariate analysis, mortality was predicted by persistence of epileptic spasms (odds ratio [OR] = 4.30, 95% confidence interval [CI] = 1.11-16.67, P = .035) and significant respiratory system comorbidity (OR = 12.75, 95% CI = 2.88-56.32, P = .001). Mortality was epilepsy-related in one-third of patients who died with sudden unexpected death in epilepsy (SUDEP), accounting for 88% of epilepsy-related deaths. Most deaths before age 5 years were related to respiratory failure, and SUDEP was less common (17%) whereas SUDEP was more common (45%) with deaths after 5 years. For the majority (67%) of patients with early mortality, an end-of-life care plan was in place (based on documentation of resuscitation status, comfort measures, or decision not to escalate medical care).
Mortality at our single-center IS cohort was 17%, and persistence of epileptic spasms and comorbid respiratory system disorders were the most important determinants of mortality. Early deaths were related to neurological impairments/comorbidities. SUDEP was more common in children who died after 5 years of age than in those who died younger than 5 years.
确定婴儿痉挛症（IS）患儿死亡的相关风险因素和病因。我们描述了死亡患儿的整体治疗目标。
这是一项对 2000 年至 2011 年出生的 IS 患儿进行的回顾性图表研究。我们检查了死亡的潜在风险因素，包括病因、神经损伤、药物使用、癫痫性痉挛持续存在以及合并系统受累（需要胃造口喂养、呼吸干预）。对于死亡患儿，我们描述了死亡原因和复苏状态或生命末期护理措施。
我们确定了 150 名 IS 患儿，中位随访时间为 12 年。在研究期间，25 名（17%）患儿死亡，其中 13 名患儿死亡年龄小于 5 岁。单因素分析表明，发育迟缓、可识别病因、IS 激素治疗、癫痫性痉挛持续存在、抗癫痫药物联合用药、难治性癫痫、呼吸系统合并症以及需要胃造口均为死亡的显著危险因素。多因素分析表明，癫痫性痉挛持续存在（比值比 [OR] = 4.30，95%置信区间 [CI] = 1.11-16.67，P =.035）和呼吸系统显著合并症（OR = 12.75，95% CI = 2.88-56.32，P =.001）是死亡的预测因素。死亡患儿中，有三分之一与癫痫相关（癫痫猝死，占癫痫相关死亡的 88%），三分之一与呼吸系统相关，另有 17%与癫痫无关。5 岁前死亡的患儿中，大部分（67%）与呼吸衰竭有关，而癫痫猝死相对较少（17%），5 岁后癫痫猝死较为常见（45%）。对于大多数（67%）早期死亡患儿，生命末期护理计划已制定（基于复苏状态、舒适护理或决定不升级医疗护理的记录）。
本单中心 IS 队列的死亡率为 17%，癫痫性痉挛持续存在和合并呼吸系统疾病是死亡的最重要决定因素。早期死亡与神经损伤/合并症有关。5 岁后死亡的患儿中，癫痫猝死的发生率高于 5 岁前死亡的患儿。
