Abstracts

Rationale: Pneumonia is a common complication and cause of death in individuals with rare epilepsy syndromes (e.g., Aicardi Syndrome). We examine the prevalence odds ratio (pOR) of risk factors for pneumonia (aspiration and/or recurrent pneumonia) across rare epilepsies. Methods: A cross-sectional study was conducted using caregiver entered data in the Rare Epilepsy Network. Caregivers entered data on their affected child, including, immune disorders, neuromuscular scoliosis, gross motor delay, aspiration of food into the lungs, G-tube usage, hyperventilation, breathing device while sleeping, and dysphagia, usage of topiramate or sedating AEDs and pneumonia. Bivariate and multivariable logistic regression were conducted for risk factors associated with a pOR for pneumonia. The Sobel mediation test was used to show that factors associated with pneumonia (e.g., neuromuscular scoliosis) were mediated by another factor (e.g., hyperventilation) and the indirect effect was calculated. Results: Of 685 rare epilepsy patients, 69 were diagnosed with one or both types of pneumonia, for a cumulative lifetime prevalence of 10.1%. 7 (10.1%) had recurrent pneumonia only, 26 (37.7%) had aspiration pneumonia only, and 36 (52.2%) had a history of both pneumonia types. All factors but acetazolamide and zonisamide were statistically significantly associated with an increased pOR for pneumonia (Table 1). In a multivariate binary logistic regression for pneumonia, hyperventilation increased the pOR of pneumonia 14.3-fold (95% CI 2.9-70.3) and aspiration of food into the lungs increased the pOR 13.4-fold (95% CI 7.1-25.3). Aspiration of food into the lungs was a significant mediator between topiramate (p=0.04) and pneumonia. Neuromuscular scoliosis was mediated by hyperventilation (p=0.02), gross motor delay was mediated by aspiration of food into the lungs (p < 0.001; Table 2). In 7 rare epilepsies, aspiration of food into the lungs, G-tube usage, dysphagia, and hyperventilation were associated with lifetime pneumonia. Conclusions: We found strong associations between immune disorders, neuromuscular scoliosis, gross motor delay, aspiration of food into the lungs, G-tube usage, hyperventilation, breathing device while sleeping, and dysphagia with pneumonia and moderate associations with usage of topiramate or sedating AEDs and pneumonia in the rare epilepsies.  This should raise physicians’ concerns about developing pneumonia among patients with rare epilepsies. Given the association of mostly neurologic problems with pneumonia in this population, it is possible that improved efforts to provide multidisciplinary care, or at least an increase in monitoring, may be an effective intervention to lower the risk for pneumonia. Further work is needed to develop protocols for interventions that target the strongest risk factors for pneumonia in the rare epilepsies in order to decrease the occurrence of pneumonia.  Funding: PCORI