Breath-Holding Spells

Basics

DESCRIPTION

  • 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

EPIDEMIOLOGY

  • Incidence: not reported
  • Prevalence: 4.6% (severe), up to 27% (simple)
  • No gender difference
  • 20–35% have a positive family history
  • Age/frequency
    • Median age of onset 6 to 12 months of age
    • Typically ages 1 to 5 years but can occur up to 7 years of age
    • Usually resolve by school age
    • Frequency
      • Can occur several times per day to only once a year
      • Age of peak frequency of spells is from 1 to 2 years of age.

RISK-FACTORS

  • 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.

GENERAL-PREVENTION

  • 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.

PATHOPHYSIOLOGY

  • 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

ETIOLOGY

Always provoked by anger, pain, or frustration

ASSOCIATED-CONDITIONS

  • 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.

There's more to see -- the rest of this topic is available only to subscribers.