Breath-Holding Spells

Basics

Breath-holding spells are the general term for emotionally provoked attacks that occur in young children. These attacks can progress from a strong emotion to “breath holding” to decreased sensorium and either limpness or stiffness, which can appear as seizure-like activity.
Disease essentials
- Provoked by anger, pain, or frustration
- Association with altered respiratory effort
- Results in decreased muscle tone
- Can be classified as simple (brief, no loss of consciousness) or severe (prolonged, associated loss of consciousness)
Subtypes
- Cyanotic (80%)
  - Classic breath-holding spells
  - Typically associated with anger
  - Progress from crying to exhalation to apnea and syncope to decreased muscle tone and falling
  - May also note generalized clonic jerks, opisthotonos, and bradycardia
  - Ages: 6 months to a peak at 2 years, with resolution by 5 years
- Pallid (20%)
  - Typically associated with pain, frustration, or surprise
  - Progress from quieting to apnea (at the end of expiration) to syncope to decreased muscle tone and falling
  - May also note clenched hands and clonic jerks and bradycardia

Underlying autonomic regulatory dysfunction
Inheritance
- 20–35% of patients with breath-holding spells have a positive family history.
- 11% of patients with epilepsy or other chronic, but nonneurologic disorder have a positive family history of breath-holding spells.
- For 80% of patients with severe spells and a positive family history, the affected family members are mainly on the maternal side.
- An autosomal dominant trait with reduced penetrance has been noted in some.

There are no known methods, medications, or treatments for preventing breath-holding spells.
Although the term breath-holding spells implies volition, these attacks are involuntary and reflexive.
For a variety of reasons, emotional outbursts are common in this age group; however, appeasing a child to prevent a spell is not recommended as it may lead the child to develop other, similar-appearing behaviors encouraging parental concession.

Cyanotic breath-holding spells
- Syncope due to a Valsalva maneuver increasing the intrathoracic pressure, decreasing cardiac blood return and eventually cardiac output, which causes cerebral hypoperfusion and unconsciousness
Pallid breath-holding spells
- Abnormal vagal response to emotional stimulation causing bradycardia and/or asystole, leading to decreased cardiac output and cerebral ischemia and unconsciousness

Always provoked by anger, pain, or frustration

No definitive associated conditions
There have been reports of some children with breath-holding spells going on to have syncope and/or seizures.
Anemia:
- Some studies have noted an increased prevalence of anemia in children with breath-holding spells; the anemia and spells improved over time with iron treatment.
- Although these findings also coincide with the expected timing for resolution of breath-holding spells, anemia might complicate an individual child’s picture.
- The suggested mechanisms for iron deficiency associated with breath-holding spells include lowered oxygen levels in the central nervous system, catecholamine disruption, and increased crying/fussiness (known trigger) with iron deficiency.
New research has found an association between the frequency of breath-holding spells and the frequency of respiratory sinus arrhythmia.
There is an increased risk of vasovagal syncope in adolescents and adults, who had pallid breath-holding spells as children.

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