Prospective Study of Children With Cyanotic and Pallid Breath-Holding Spells

Abstract

Objective. In this investigation, we sought to prospectively document the natural history of severe breath-holding spells (BHS) among children with both cyanotic and pallid BHS who were referred for neurologic consultation. Design. Prospective cohort study. Methods. A structured interview was undertaken at the time of initial consultation and at subsequent 1-year intervals regarding type of BHS, frequency of spells, associated phenomenon, sequelae, family history, and age at termination of spells. Results. A total of 95 children (48 boys, 47 girls) with BHS were identified and followed over a 9-year interval. There were no significant differences between genders. Median onset age was between 6 and 12 months old with 15% presenting younger than 6 months. A median frequency of spells was weekly with 30% experiencing 1 or more spells per day. The median age at peak frequency was between 12 and 18 months old with a range extending from 6 months to 4 years of age. Of the patients whose BHS had remitted for >12 months' time (n = 67), the last spell occurred at a median age of 37 to 42 months. Of those children whose BHS were still occurring, the oldest age at time of latest spell was at 7 years old. Hypoxic convulsions were associated with BHS in ≤15% of all participants. A positive family history of BHS was identified in 34% of all families with equal frequency distributed between paternal and maternal sides. Conclusions. In this study, new data concerning the natural history of BHS have been determined. The information is important for family counseling and identifying intervention strategies, and serves as baseline data to evaluate the efficacy of future treatment approaches.