Breath-holding spells are the occurrence of episodic apnea in children, possibly associated with loss of consciousness, and changes in postural tone. Breath-holding spells occur in approximately 5% of the population with equal distribution between males and females. They are most common in children between 6 and 18 months and usually not present after 5 years of age. They are unusual before 6 months of age. A positive family history can be elicited in 25% of cases. They may be confused with a seizure disorder. They are sometimes observed in response to frustration during disciplinary conflict.
Diagnosis
Classification
There are four types of breath-holding spells.
The most common is termed simple breath-holding spell, in which the manifestation is the holding of breath in and expiration. The usual precipitating event is a frustration or injury leading to the temporary cessation of breathing in and expiration. There is no major alteration of circulation or oxygenation and the recovery is spontaneous.
The second type are the Cyanotic breath-holding spells. They are usually precipitated by anger or frustration although they may occur after a painful experience. The child cries and has forced expiration sometimes leading to cyanosis, loss of muscle tone, and loss of consciousness. The child usually recovers within a minute or two, but some fall asleep for an hour or so. Physiologically, there is often hypocapnea and usually hypoxia. The events are thought to occur due to a variety of factors, including the fact that the child is not breathing, there is increased intrathoracic pressure secondary to the Valsalva maneuver, and decreased cardiac output. This eventually leads to a significant decrease of circulation to the brain and ultimately, loss of consciousness. There is no postictal phase, no incontinence, and the child is fine in between spells. EEGs are normal in these children. There is no relationship to the subsequent development of seizures or cerebral injury as a consequence of breath holding spells.
In the third type, known as Pallid breath-holding spells, the most common stimulus is a painful event. The child turns pale and loses consciousness with little if any crying. The EEG is also normal, and again there is no postictal phase, nor incontinence. The child is usually alert within a minute or so. There may be some relationship with adulthood syncope in children with this type of spell.
A fourth type, known as Complicated breath-holding spells, may simply be a more severe form of the two most common types. This type generally begins as either a cyanotic or pallid spell that then is associated with seizure like activity. An EEG taken while the child is not having a spell is still generally normal.
The diagnosis of a breath-holding spell is made clinically. A good history including the sequence of events, lack of incontinence and no postictal phase, help to make an accurate diagnosis. Some families are advised to make a video recording of the events to aid diagnosis. An electrocardiogram may rule out cardiac arrhythmia as a cause. There is some evidence that children with anemia may be more prone to breath-holding spells.
Treatment
The most important approach is to reassure the family, because witnessing a breath-holding spell is a frightening experience for observers. There is no definitive treatment available or needed for breath-holding spells, as the child will eventually outgrow them. Some trials have demonstrated the efficacy of iron therapy, especially because although BHS can readily occur without anemia, BHS has been found to be exaggerated by the presence of anemia. Other studies have supported the use of piracetam; a 1998 study indicating that over two months piracetam reduced BHS incidence by sixty percent, twice as much as a placebo. All of these studies agree with the established medical view that a pharmacological agent is not necessary, although it may be desirable for the comfort of the parent and child. Two articles on breath-holding spells strongly suggest that parents consider having their child be tested by electrocardiogram for the rare, but real possibility that the BHS episodes are actually a symptom of prolonged QT-syndrome, a serious but treatable form of cardiac arrythmia.
