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Mortality in infantile spasms: A hospital-based study
Chellamani Harini1, Elanagan Nagarajan1, Ann M Bergin1
1Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, Massachusetts.
Insights
Infantile spasms (IS) mortality is 17%, with persistent spasms and respiratory issues being key risk factors. Early deaths link to neurological issues, while later deaths show higher SUDEP rates.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Research
Background:
- Infantile spasms (IS) is a severe epilepsy syndrome in infants.
- Understanding mortality risk factors in IS is crucial for patient management and care planning.
Purpose of the Study:
- To identify risk factors and causes of mortality in children with infantile spasms.
- To describe end-of-life care goals for deceased IS patients.
Main Methods:
- Retrospective chart review of 150 IS patients born between 2000-2011.
- Analysis of potential mortality risk factors including etiology, neurological impairment, medication, spasm persistence, and comorbidities.
- Review of cause of death and end-of-life care for deceased patients.
Main Results:
- Overall mortality was 17% (25/150), with 13 deaths before age 5.
- Multivariate analysis identified persistent epileptic spasms (OR=4.30) and respiratory comorbidity (OR=12.75) as significant predictors of mortality.
- Epilepsy-related deaths accounted for one-third of mortality, with Sudden Unexpected Death in Epilepsy (SUDEP) being more common in older children.
- Respiratory failure was the primary cause of death in younger children (<5 years).
Conclusions:
- Persistent epileptic spasms and respiratory comorbidities are major determinants of mortality in IS.
- Mortality patterns differ by age, with neurological impairments/comorbidities dominating early deaths and SUDEP increasing in older children.
- End-of-life care plans were documented for the majority of early mortality cases.
Objective:
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.
Methods:
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.
Results:
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).
Significance:
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.
