Objective: To examine the incidence and risk factors for death among neonates who developed neonatal seizures (NS) in a culturally and ethnically distinctive community with high consanguinity rate. Methods: Retrospective study conducted at a single institution on data between January 2001 and January 2016. All neonates diagnosed with NS developed up to age 28 days were included. Mortality was defined as death within the first year of life. Data was obtained from the hospital discharge register with ICD-9 codes and crosschecked with the labor medical records. The primary outcome examined was risk factors for death in the first year of life associated with NS. Results: Of all 69,460 neonates born during the study period, 118 (1.7 per 1000 live births) developed NS; 35 (29.7%) died within the first year and comprised the deceased group, 83 (70.3%) survived and comprised the survived group. Any consanguinity between parents was found in 18% and 14.6% among the survived and deceased groups, respectively (P=0.24). Anatomical disorders that lead to death were present in 3.6% and 31.4% in the survived and deceased groups, respectively (P=0.001; RR 8.70; 95%CI 2.58-29.27). Stepwise backward logistic regression analysis revealed that anatomical cause for seizures (P<0.0001), use of more than one anti-epileptic medication (P=0.006), and multiorgan failure (P=0.004) were significant risk factors that predicted death. Conclusion: Anatomical cause for seizures was a significant risk factor that led to death. This is probably related to the relatively high consanguinity rate of the culturally and ethnically distinctive community examined
