Breath-Holding Spells
                        =====================
þ Breath-holding classification:
 - two of the various kinds of anoxic non-epileptic seizures of early
   childhood.
 - NOT related to voluntary breath-holding, which is inspiration; cyanotic
   breath-holding spells are in expiration.
 - usually the actual seizures are short, 6-8 seconds in scientific studies
   (done before there were IRBs), and occur during reperfusion of the brain.

þ Two kinds:
 - pallid:
  + excessive vagal stimulus to heart causes bradycardia or temporary asystole
  + breath-holding per se is a minor part of this.
  + usually from a sudden, unexpected and usually painful stimulus, such as a
    blow to the head
  + has been reproduced by pressure on the eyeballs
 - cyanotic
  + complex pathogenesis: hyperventilation, Valsalva maneuver, expiratory
    apnea, others.
  + usually from infant/toddler getting very angry or upset

þ Typical Cyanotic Breath-Holding Spell (BHS)
 - Ages 6 months - 2 years (rarely onset up to age 4)
 - kid gets angry, holds breath, turns blue, passes out, briefly stiff but no
   true seizure
 - sometimes has a brief true seizure
 - expiratory apnea > color change > LOC
 - need detailed history

þ Natural history (pallid and cyanotic both)
 - usually starts in first 18 months of lifxe, 90% or more before age 2
 - go away by age 4 in half of patients, by age 7-8 in almost all patients
 - may be predisposed to syncope as an adult; otherwise no sequelae
 - may be family history
 - fever or anemia may make BHS more likely
 - unless other severe illness, sudden death very, very unlikely
 - seizures always brief, unless underling epilepsy

þ Prolonged QT Syndrome
 - may mimic breath-holding precisely
 - ALWAYS get an EKG and check the QT interval

þ Evaluation of Breath-Holding Spells
 - Get an EKG.
 - Don't admit
 - If cyanotic, don't do any other studies
 - If possibly pallid, refer to neurologist for EEG/EKG with ocular pressure to
   look for EEG slowing/asystole.
 - If prolonged, work up as for epileptiform seizure

þ Treatment of Breath-Holding Spells
 - Basically, nothing.
 - Not worth trying to avoid causative events--just not possible without
   damaging child development.
 - Worth teaching family:
  + NO CPR unless lasts for longer than a minute or two (one report of death
    likely from CPR-related aspiration pneumonitis)
  + Use recovery/coma position for airway protection
 - If frequent and persistent, few case reports of
  + treating with atropine or methonitrate
  + treating with a pacemaker
  + treating with transdermal scopolamine
 - Seizure medications not helpful, sometimes harmful

Breningstall, G. N. (1996). "Breath-holding spells." Pediatr Neurol 14(2):
91-7.
        Two particularly common, and frequently frightening, forms of syncope
and anoxic seizure in early childhood are pallid and cyanotic breath-holding
spells. Pallid breath-holding spells result from exuberant vagally-mediated
cardiac inhibition. Cyanotic breath-holding spells are of more complex
pathogenesis, involving an interplay among hyperventilation, Valsalva
maneuver, expiratory apnea, and intrinsic pulmonary mechanisms. The history is
the mainstay of diagnosis; videotape documentation may be possible.
Performance of an electrocardiogram to evaluate for prolonged QT syndrome
should be strongly considered. In patients with pallid breath-holding spells,
a characteristic sequence of changes may be documented on an
electroencephalogram with ocular compression, if this study is performed.
Spontaneous resolution of breath-holding spell, without sequelae, is
anticipated. Reassurance is the mainstay of therapy. Occasionally,
pharmacologic intervention may be of benefit.