This study attempted to determine noninvasively whether a dysregulatio
n of autonomic nervous system reflexes exists in children with severe
cyanotic breath-holding spells (BHS). This was a cross-sectional study
performed in the neurophysiology laboratory at a tertiary care hospit
al. Patients, 18 months of age and older taken from a referral populat
ion of children with severe cyanotic BHS and normal controls, were stu
died. Fourteen children with cyanotic BHS and 12 controls were evaluat
ed. Several noninvasive measures of parasympathetic and sympathetic re
flex functions were assessed and statistically analyzed using analysis
of variance and covariance, and Fisher's exact tests. The cyanotic BH
S group had a significantly greater increase in pulse rate at 15 s of
standing after rising from the supine position (P < .06), with a trend
toward a concomitant higher mean arterial pressure (P < .09). After a
djusting for age, sex, and mean supine systolic and diastolic blood pr
essures, breath-holders had a greater decrease in diastolic blood pres
sure (P < .02) without an increase in systolic blood pressure after st
anding from the supine position. Breath-holders also had significantly
abnormal 30:15 R-R interval ratios compared with controls (P < .002).
These results support the hypothesis that children with cyanotic BHS
have underlying autonomic nervous system dysregulation. This dysregula
tion may contribute to the pathophysiology of severe BHS in these chil
dren.