In a breath-holding spell (BHS) a child appears to hold his or her breath, becomes pale or blue, and then, if severe, loses awareness. It is a reflex, involuntary act, although it may result from a voluntary or semi-voluntary act causing the child to become emotional. Typically, a BHS begins with a provoking event, such as frustration, surprise, anger or fear. Crying or whimpering ensues and then pauses, at which time, a facial color change is evident. If the episode persists, the child will become poorly responsive, and may lose consciousness, becoming limp and falling. The term “breath-holding” causes confusion, as it may suggest that children hold their breath on purpose. This is not the case. Breathing may stop in some BHS, but the children do not hold their breath voluntarily.

The prevalence of BHS ranges from 0.1% to over 4% in different studies (DeMario 1999). The condition has a mild tendency to run in families. Onset is before age 2 years in over 90% (Breningstal 1996). Rate of attacks is highly variable: occurring only once in a lifetime or multiple times per day. BHS usually resolve without treatment by age 4, but sometimes persist to age 8 years. Another important condition with loss of consciousness is called “reflex anoxic seizures”; however, this is believed by many to be distinct from BHS. Information on reflex anoxic seizures can be found at www.stars.org.uk.

BHS come in two varieties. The first is associated with a pale, white facial color during the episode, suggesting decreased blood flow. The medical term for this type is “pallid” BHS. With the second type, a blue of purple facial color is observed, suggesting decreased oxygen. The medical term for the blue form is “cyanotic.” A typical description was given by Stephenson in 1978: “An unsteady toddler on his own trips and falls. His mother hears the bump but no succeeding cry and hurries to him. She finds her child lying deathly still with eyes fixed upwards, lips dusky. As she lifts him, he abruptly stiffens into rigid extension with jaw clenched and hands fisted, gives a few jerks, and after what seems an age (but in fact is less than half a minute) relaxes limply with an absent far-away look. Then he opens his eyes, at once recognizes his mother, cries a little, and drifts off to sleep, his face distinctly pale.”

Some jerking movements and loss of bladder control can accompany a BHS, imitating an epileptic seizure. While this event is actually a seizure, the seizure is a reaction to the brain temporarily not receiving sufficient oxygen and blood flow, not to a primary epileptic discharge. A characteristic sequence of inciting stimulus, a cry, pause, facial color change, limp unresponsiveness and then jerking marks it as a BHS, not epilepsy.

Breath-holding spells need to be distinguished from epilepsy, typical fainting episodes (medically called syncope), from gastrointestinal acid reflux in babies, sleep apnea, cardiac heartbeat irregularities (arrhythmias) and temper tantrums. The best way to distinguish epilepsy from breath-holding is to search for a trigger: epilepsy rarely has one and breath-holding almost always does. Temper tantrums do not themselves lead to loss of consciousness, unless they are a trigger for a BHS. Electroencephalograms (EEGs, brainwaves) sometimes show abnormalities between the episodes that support a diagnosis of epilepsy over BHS. EEGs usually are normal in children with BHS, but they also can be normal between seizures in children with epilepsy. Therefore, only an abnormal EEG with changes suggestive of epilepsy is of value for distinguishing these conditions.

The cause of BHS is not known. Some attacks result from hyperactive normal reflexes, such as slowing of the heart rate when the eyes are rubbed, or when the child tries to breathe or scream against a closed throat (the Valsalva response). The children may have hyperactivity of the normal protective responses that the body automatically activates when oxygen levels begin to fall.

Prognosis of BHS is very good, but it must be said that rare cases of injury have been reported. Treatment consists of reassurance, and recognition that the episodes are rarely in the child’s control. In the pale form, with demonstrated slow heartbeat, medications such as scopolamine have been used to block the slowing of the heartbeat. A few children with BHS have significant anemia and low amounts of iron in the blood, limiting the oxygen carrying capacity of blood. These may be successfully treated with iron, although scientific evidence that it helps is very limited. Ultimately, time solves the problem, since breath-holding spells are outgrown by mid-childhood.

Citations

1. Francis J. DiMario, Jr, MD . Breathholding spells in childhood. Curr Probl Pediatr, November/December 1999, 281-299.
2. Breningstall GN. Breath-holding spells. Pediatr Neurol 1996; 14:91-97.
3. Stephenson JBP. Reflex anoxic seizures ('white breath-holding'): Nonepileptic vagal attacks. Arch Dis Child 1978;53:193-200.

by Robert S. Fisher, M.D., Ph.D., J. Gert van Dijk, M.D., Ph.D., Trudie Lobban