Pediatric Annals

Breath-Holding Spells

Owen B Evans, MD

Abstract

Breath-holding spells, also called syncopal attacks, are episodes consisting of apnea, unconsciousness, and changes in postural tone triggered by adverse stimuli. Tonic, clonic, or tonicclonic activity may or may not accompany the episode. Breath-holding spells have been discussed in the medical literature for several hundred years. Almost all of the early pediatric textbooks included descriptions of the phenomena. Several large studies have documented the epidemiology, course, prognosis, and occasionally treatment of breath-holding spells.1"3 More recent reports have added to the understanding and management of one of the most common episodic disorders of childhood.

In addition to the breath-holding spell, there are a number of other episodic disorders in infants and children that enter into the differential diagnosis of seizures and epilepsy. Some of these, like breathholding spells, appear to be provoked by a stimulus. These and related spells are discussed in this review.

EPIDEMIOLOGY

Much of information on the epidemiology of breath-holding spells comes from a large prospective study that was a part of the Maternal Infant Health Collaborative Project.1 Over a 2-year period between 1963 and 1965, 4980 infants in the Boston area were entered into the system and followed up from birth to 8 years. Mothers were questioned regarding the occurrence of breath-holding spells. Two hundred twenty-five infants (4-6%) were identified, and information about them was compared with that from 384 random control infants. The same authors included 83 children from a retrospective study of patients with the diagnosis of breath-holding spells.

The incidence in males and females is almost the same, with a slight male predominance. There are no socioeconomic differences between children with breath-holding spells and control subjects. Nor is there a significant increase in the incidence of mental retardation, epilepsy, or other neurologic diseases.1,3 Approximately 25% of infants with breath-holding spells have a positive family history of similar episodes compared with 11% of control subjects.3 Eighteen percent of mothers wirh children who have breathholding spells reported abnormal behaviors such as excessive stubbornness, disobethence, or aggression. These behaviors were reported in 8% of control patients.1

The vast majority of infants have onset of breathholding spells between 6 and 18 months. The onset is rare after 2 years of age, and there has not been a welldocumented case of breath-holding spells beginning after 4-5 years of age.2 Breath-holding spells have been reported in the first weeks of life, although some authors have disputed whether these reports represent breath-holding spells or aspiration.3 Approximately 7% of children with breath-holding spells have onset during the first month. The duration is quite variable, but 90% of children with breath-holding spells no longer have them by 6 years of age. The frequency of the episodes vary considerably. Typically they are infrequent at first, become increasingly more frequent after several months, and then taper off. The majority of affected children have at least one episode per week or more.

CLASSIFICATION

Breath-holding spells can be divided into two large types - cyanotic and pallid.1 Most children have one or the other, but a small percentage have both. Each of these in turn can be described as being either simple or complicated depending on the absence or presence of seizure-like activity.

Cyanotic Breath-Holding Spells

Approximately 60% of children with breathholding spells have cyanotic breath-holding spells. The sequence of the event is somewhat characteristic for all affected children. The event begins with a stimulus. In 72% of children with cyanotic breathholding spells, the stimulus is usually anger or frustration and less often pain or other stimuli. Fifty-four percent of children have only a single stimulus that repeatedly provokes the breath-holding spell. A spell occurs spontaneously only…

Breath-holding spells, also called syncopal attacks, are episodes consisting of apnea, unconsciousness, and changes in postural tone triggered by adverse stimuli. Tonic, clonic, or tonicclonic activity may or may not accompany the episode. Breath-holding spells have been discussed in the medical literature for several hundred years. Almost all of the early pediatric textbooks included descriptions of the phenomena. Several large studies have documented the epidemiology, course, prognosis, and occasionally treatment of breath-holding spells.1"3 More recent reports have added to the understanding and management of one of the most common episodic disorders of childhood.

In addition to the breath-holding spell, there are a number of other episodic disorders in infants and children that enter into the differential diagnosis of seizures and epilepsy. Some of these, like breathholding spells, appear to be provoked by a stimulus. These and related spells are discussed in this review.

EPIDEMIOLOGY

Much of information on the epidemiology of breath-holding spells comes from a large prospective study that was a part of the Maternal Infant Health Collaborative Project.1 Over a 2-year period between 1963 and 1965, 4980 infants in the Boston area were entered into the system and followed up from birth to 8 years. Mothers were questioned regarding the occurrence of breath-holding spells. Two hundred twenty-five infants (4-6%) were identified, and information about them was compared with that from 384 random control infants. The same authors included 83 children from a retrospective study of patients with the diagnosis of breath-holding spells.

The incidence in males and females is almost the same, with a slight male predominance. There are no socioeconomic differences between children with breath-holding spells and control subjects. Nor is there a significant increase in the incidence of mental retardation, epilepsy, or other neurologic diseases.1,3 Approximately 25% of infants with breath-holding spells have a positive family history of similar episodes compared with 11% of control subjects.3 Eighteen percent of mothers wirh children who have breathholding spells reported abnormal behaviors such as excessive stubbornness, disobethence, or aggression. These behaviors were reported in 8% of control patients.1

The vast majority of infants have onset of breathholding spells between 6 and 18 months. The onset is rare after 2 years of age, and there has not been a welldocumented case of breath-holding spells beginning after 4-5 years of age.2 Breath-holding spells have been reported in the first weeks of life, although some authors have disputed whether these reports represent breath-holding spells or aspiration.3 Approximately 7% of children with breath-holding spells have onset during the first month. The duration is quite variable, but 90% of children with breath-holding spells no longer have them by 6 years of age. The frequency of the episodes vary considerably. Typically they are infrequent at first, become increasingly more frequent after several months, and then taper off. The majority of affected children have at least one episode per week or more.

CLASSIFICATION

Breath-holding spells can be divided into two large types - cyanotic and pallid.1 Most children have one or the other, but a small percentage have both. Each of these in turn can be described as being either simple or complicated depending on the absence or presence of seizure-like activity.

Cyanotic Breath-Holding Spells

Approximately 60% of children with breathholding spells have cyanotic breath-holding spells. The sequence of the event is somewhat characteristic for all affected children. The event begins with a stimulus. In 72% of children with cyanotic breathholding spells, the stimulus is usually anger or frustration and less often pain or other stimuli. Fifty-four percent of children have only a single stimulus that repeatedly provokes the breath-holding spell. A spell occurs spontaneously only very rarely, and cyanotic breath-holding spells never occur during sleep.

After a short period of vigorous crying, breathing is interrupted during expiration. The child becomes cyanotic initially around the lips, becomes limp, and then loses consciousness for a brief period of time. Most parents have observed this "waiting to inhale"4 phenomenon in vigorously crying infants. Approximately one half will return to consciousness immediately and are asymptomatic, whereas the remainder may sleep for a variable period of time, but rarely longer than an hour or so.

The pathophysiology of cyanotic breath-holding spells is probably related to a number of physiologic events.1 The violent crying causes hypocapnia with resulting cerebral ischemia. Cessation of breathing causes hypoxemia, and the Valsalva maneuver, when the child is in respiratory spasm during expiration, causes increased intrathoracic pressure and reduced cardiac output. All of these factors subsequently cause impaired cerebral circulation with loss of consciousness as the result. It has been likened to the "mess trick."5 This is a favorite adolescent stunt in which one hyperventilates and then performs a Valsalva maneuver. This causes an altered state of consciousness and euphoria. It has been speculated that tussive syncope and micturition syncope have a similar pathophysiology. Some children, perhaps all, with cyanotic breath-holding spells have similar autonomic disturbances as described for children with pallid breath-holding spells.4

It is unclear whether there is a manipulative component to breath-holding spells or not. Children with severe breath-holding spells do not show significant differences from normal children on standardized behavioral scores.6 More likely, children with cyanotic breath-holding spells represent a subset of the population that is more sensitive to cerebral ischemia than others. Cyanotic breath-holding spells occur most frequently during the "terrible two's," a period of time when anger and frustration are not easy for the child to deal with. This obviously does not explain the cases in younger infants and older children.

The prognosis for cyanotic breath-holding spells is excellent. There is no evidence on long-term follow up that the recurrent episodes of loss of consciousness cause cerebral injury.1,3 Long-term studies have shown no difference in the results of psychometric testing between children with breath-holding spells and control subjects. There is also no evidence of a higher incidence of later epilepsy.

Pallid Breath-Holding Spells

In the classic paper by Lumbroso and Lerman,1 approximately 20% of children who had breathholding spells had a very rapid loss of consciousness with little or no crying. As opposed to cyanosis, pallor was the major clinical feature before and during the period of altered consciousness. Pain is by far the most common stimulus that causes pallid breathholding spells; anger or fear is the stimulus much less often. Interestingly, unlike in older children or adults, the sight of blood is not recognized as a common stimulus for pallid breath-holding spells in infants.2 The period of altered consciousness and the subsequent recovery is very similar to that in cyanotic breathholding spells.

The pathophysiology of pallid breath-holding spells, however, appears to be distinctly different. Children with pallid breath-holding spells have a period of asystole from one to several seconds just after the stimulus and just before the altered consciousness. They can also be shown to have an exaggerated oculocardiac reflex. Pressure on the globes of the eyes can elicit asystole in 61% of children with pallid breath-holding spells but in only 25% of children with cyanotic breath-holding spells.1 Incidentally, the use of pressure on the eyes was a diagnostic test for pallid breath-holding spells as to revert supraventricular tachycardia may carry some risks of retinal detachment and many recommend against this procedure. The longer the period of asystole, the more likely is the development of more complicated features of the breath-holding spell. Autonomic regulation of the cardiovascular system is abnormal in some children with pallid breath-holding spells. There is a greater fall in the diastolic blood pressure without an increase in systolic pressure after standing and an abnormal 30:15 R-R interval ratio.7 Further evidence that pallid breath-holding spells are associated with autonomic dysfunction comes from long-term follow-up studies. Approximately 15% of children with pallid breath-holding spells later have syncope as young adults.3 A dominant autosomal transmission with reduced penetrance has been recently documented in a large kindred.8

Complicated Breath-Holding Spells

Complicated breath-holding spells are defined as typical breath-holding spells followed by seizure-like activity. Generally speaking, the more prolonged the period of unconsciousness, the more likely the development of convulsive activity; however, this is not always the case. Approximately 15% of patients with breath-holding spells have complicated features.1,3 Clonic activity appears as consciousness is lost. With prolonged loss of consciousness, there follows generalized stiffening with either decerebrate or decorticate posturing. Often following this phase is another period of clonic activity then relaxation and features resembling a post-ictal state. Most complicated breath-holding spells are associated with just the tonic posturing. Loss of bladder control is rare and only occurs in patients who have tonic posturing.

Electroencephalography (EEG) recordings of children during complicated breath-holding spells show similar features.1 Simultaneous with the early hypoxia, the normal background rhythms become hypersynchronous. As the spell continues, rhythmic slow activity is recorded, which coincides with early clonic activity. Then follows a brief period of electrocerebral silence simultaneous with the loss of consciousness followed again by synchronous delta activity with slow recovery to the normal rhythms. Epileptiform "spike" discharges are not recorded, and the interictal EEG result is almost universally normal with only occasional nonspecific findings. The veni-puncture fit is a similar disorder.9

DIAGNOSIS

The diagnosis is self-evident in most cases because of the predictable relationship of the provoking stimulus, the behavioral response, and the subsequent loss of consciousness. Because the physician rarely has the opportunity to actually observe the spell, a careful history is the key to diagnosis. When the diagnosis is unclear from the history, a video recording of the spells should be made by the parents. There is no specific diagnostic test for breath-holding spells. A number of other spells and related phenomena should be considered in the differential diagnosis of breathholding spells.

Epilepsy is recurrent, unprovoked seizures. Often, breath-holding spells, especially complicated ones, are confused with epilepsy. The Table is a useful guide for distinguishing the two. The seizures of epilepsy occur more or less randomly but are prone to occur with fever and infection, from lack of sleep, and during sleep. Aside from a few rare reflex epilepsies, the seizures of epilepsy do not predictably follow a stimulus. Generalized epilepsy is characterized by generalized motor or absence seizures. The ictal event during a generalized motor seizure is tonic or clonic muscle activity or a combination of the two, and the post-ictus is one of sleep. Minor motor seizures are brief alterations in postural tone, such as myoclonus or atonus, which can cause frequent falling (drop attacks). Absence seizures are brief interruptions in awareness. Partial epilepsy is either simple or complex. Simple partial epilepsy consists of recurrent partial seizures without alteration in consciousness. Symptoms are most often motor with involuntary movement of the face or an extremity. Sensory and other simple seizures can occur, but the description of the event is difficult for a child. The post-ictus may show Todd's paralysis. Complex partial epilepsy consists of recurrent complex partial seizures in which there is an alteration of awareness or behavior as a key component of the ictal event. Automatisms such as sucking or chewing may occur. The post-ictus is often one of confusion or sleep.

Table

TABLEClinical Features of Seizures and Breath-Holding Spells

TABLE

Clinical Features of Seizures and Breath-Holding Spells

Impact seizures occur in toddlers after blows to the head. Most often these seizures follow a fall or result from the child hitting the head on countertops and other obstacles. The seizure is immediate and resembles a typical generalized tonic-clonic seizure followed by a post-ictal state. This is usually the only situation in which the child has a seizure. Whether this type of seizure represents a special type of early posttraumatic seizure or a variant of complicated breath-holding spells is unknown. Immersion seizures occur with bathing the head of infants and not in older children. The pathophysiology is unknown.

The temper tantrum results from the conflict arising from limits set by the parents to the child's desire for autonomy. The tantrum is one mechanism a child uses to gain control, and it becomes a successful strategy if it is intermittently successful by causing the parent to yield. Tantrums begin toward the end of the first year, peak during the second year, and usually resolve by 4 years of age, a pattern coinciding very closely with the risk years for breath-holding spells. Certain infants are more prone to tantrums than others, especially those who are very active and who respond intensely to conflict. Tantrums arise when the infant or child tests limits. The toddler desires something (or does not), wants to leave (or does not), or cannot master a task and becomes frustrated. Fear, fatigue, lack of sleep, illness, and drugs, especially sedatives, make a child more prone to tantrums. One might consider the cyanotic breath-holding spell to be a complicated temper tantrum. Tantrums can arise from a variety of situations, whereas most breathholding spells follow a predictable stimulus. Tantrums lasting longer than 15 minutes, occurring more than three times a day, or persisting beyond 5 years of age should raise suspicions of underlying psychological or social problems.10

Rage attacks are another unusual spell in children.11 Unlike breath-holding spells or temper tantrums, rage attacks are unprovoked or follow minimal frustrations. It is more common in toddlers and preschoolaged children. For no apparent reason, the child becomes enraged and is physically or verbally abusive. The vehemence of the spell separates the rage attack from the simple temper tantrum. Also, the tantrum is a manipulative behavior, whereas the rage attack is usually unexplainable. The child is inconsolable and continues with the effort until exhausted. The child usually sleeps after the spell and may express remorse over the episode. Unlike night terrors, which this spell can somewhat resemble, rage attacks occur when the child is awake and seemingly happy. Rage attacks also occur in adults, often following brain injury. Occasionally, temporal lobe epileptiform activity can be recorded on EEG during rage attacks, suggesting that these attacks may be a seizure disorder.

Panic attacks usually begin between 7 and 14 years of age and are characterized by the sudden onset of severe anxiety for no apparent reason.12 The attack usually lasts for 10 to 30 minutes, and there are symptoms of palpitations, dizziness, dyspnea, and parenthesis. The panic attack resembles hyperventilation episodes.

In unusual circumstances, one must consider Munchausen syndrome by proxy in the differential diagnosis for spells.13 Munchausen syndrome by proxy is a disease of the parent in which there is a compulsive need for the child to have an illness. Common neurologic symptoms include convulsions, apnea, and other paroxysmal disturbances that cannot be witnessed by physicians or nurses. Shopping from physician to physician or hospital to hospital is the rule, and the parent is usually quite cooperative and seemingly a model parent. This is a serious disorder and a form of child abuse but often a diagnosis of exclusion. Malingering by proxy occurs when there is an opportunity for financial gain for the child's illness. Seizures, apnea, and other spells are frequent diagnoses.

If the type or etiology of spells is uncertain, a video recording by the parents is very useful. It is probably not wise to try and provoke a spell in the office. If the child is having very frequent episodes, simultaneous EEG and video recordings may distinguish breathholding spells from seizures. It is recommended for any child who has spontaneous spells in addition to provoked ones. If any pharmacologic therapy is being considered, an EEG should be obtained. Video recordings during a hospitalization is often needed to diagnose Munchausen syndrome or other strange events.

MANAGEMENT

Management of either type of breath-holding spell is difficult. Simple cyanotic breath-holding spells are treated with reassurance and "tincture of time." Trying to avoid the behavioral situations that typically provoke the spell is almost impossible given the developmental age of the population involved. If abnormal parenting that might be contributing to the provocation is identified, it should be corrected with counseling. One should not advise the parent to ignore the spell because they are probably caused by biologic factors and not necessarily behavioral. Some parents have reported that sprinkling cold water on the child's face will startle the child out of the spell.

Atropine has been recommended but has not been scientifically tested for the treatment of pallid breathholding spells. The dose is 0.01 mg/kg/day in two divided doses; however, the oral preparation is not available. An equivalent dose of belladonna can be used. An association between anemia and breathholding spells was noted 30 years ago.14 Recently, a clinical trial showed that iron therapy was effective in the treatment of breath-holding spells, especially in children who were iron deficient.15

The management of complicated breath-holding spells is very problematic. Breath-holding spells are frightening enough for most parents, but episodes that look like seizures often result in emergency department visits. The first time it occurs at the daycare center usually causes a "full court press." It is my practice to give a trial of anticonvulsants to children with frequent complicated spells. Carbamazepine at a dose of 10 to 20 mg/kg/day in two to three divided doses appears to be beneficial in some children. Whether the improvement is the result of antiepileptic activity or the leveling effect it has on behavior is unclear. The drug is also useful for rage attacks.

REFERENCES

1. Lombroso CT, Lerman P. Breathholding spells (cyanotic and pallid infantile syncope). Pediatrics. 1967;39:563-581.

2. Laxdal T, Gomez MR, Reiler ]. Cyanotic and pallid syncopal attacks in children (breathholding spells). Dev Med Child Neurol. 1 969; 1 1:755-763.

3. Livingston S. Breathholding spells in children: differentiation from epileptic attacks. JAMA. 1970:212:2231-2235.

4. Harmon DW. Breath-holding spells: waiting to inhale, waiting for systole, or waiting for iron therapy. J Pedum. 1977;130:510-512.

5. Howard P, Leathart GL, Domhorst AC. The 'mess trick' and the 'fainting lark.' BMJ. 1951;2:382-384.

6. DiMario FJ, Burleson JA. Clinical and laboratory observations. Behavior profile of children with severe breath-holding spells. J Pediatr. 1993;122:488-491.

7. DiMario FJ, Chee CM, Berman PH. Pallid breath-holding spells: evaluation of the autonomic nervous system. CIm Pedina. 1990;29:17-24.

8. DiMario F], Sarfarazi M. Family pedigree analysis of children with severe breathholding spells. J Pediaer. 1977;130:647-651.

9. Roddy SM, Ashwal S, Schreider S. Venipuncture fits. A form reflex anoxic seizure. Pediatrics. 1983;72:715-717.

10. Needlan RD. Growth and development. In: Behrman RE, Kliegman RM, Arvin AM, eds. Nelson Textbook of Pediatrics. 15th ed. Philadelphia, Pa: WB Saunders Co; 1996:55.

11. Elliott FA. The episodic dyscontrol syndrome and aggression. Neurol CIm. 1984;2:13-20.

12. Herkowitz J. Neurologic presentation of panic disorders in childhood. Dev Med Cfuid Neurol. 1986;28:617-623.

13. Rosenberg DD. Web of deceit: a literature review of Munchausen syndrome by proxy. Child Abuse Negl;. 1987;11:547-553.

14. Holowach J, Thurston DL. Breath holding spells and anemia. N Engl J Med. 1963:268:21.23.

15. Daoud AS, Batieha A, Al-Sheyyab M, Hijazi S. Effectiveness of iron therapy on breath-holding spells. J Pediarr. 1997;130:547-550.

TABLE

Clinical Features of Seizures and Breath-Holding Spells

10.3928/0090-4481-19970701-05

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