Mortality in infantile spasms: A hospital‐based study

Abstract

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.

Classifying etiology of infantile spasms syndrome in resource‐limited settings: A study from the South Asian region

“…It is well established that etiology unknown or cryptogenic West syndrome is associated with better response to therapy, lesser number of relapses, better developmental outcome, and a possible mortality benefit. 13 , 14 Therefore, limitations for detailed investigation are an impediment to a clear understanding of the patient's prognosis. In fairness to the huge discrepancy of resources across the globe, it seems appropriate that a dialogue is generated on how etiology can be classified “adequately” by a pediatric neurologist anywhere in the world.…”

Epilepsy and Neurodevelopmental Outcomes in a Cohort of West Syndrome Beyond Two Years of Age

“…West syndrome (WS) is a rare age-dependent epilepsy syndrome characterized by epileptic spasms (ES) in clusters, hypsarrhythmia on interictal electroencephalography (EEG), and is often associated with developmental delay or regression [1]. It is usually associated with a poor neurocognitive outcome, with early death (in one-third of patients) or frequent progression into other epilepsy syndromes [2][3][4][5][6]. The children with unknown etiology (previously referred to as cryptogenic ones) usually fare better in seizure and cognitive outcomes [2,3].…”

Brain Magnetic Resonance Imaging Findings in Infantile Spasms

“…Five percent of this cohort unfortunately died. Premature death in infantile spasms was reported to be between 5 and 30% in different studies [ 30 , 31 ]. Another observation was the high percentage of consanguinity in this cohort (59%), which is similar to the reported consanguinity numbers of 52.1–67.7% in Saudi Arabia [ 32 ].…”