2. INTRODUCTION
• BHS is a psychosomatic disorders seen commonly in children between 6 months to 6 years.
• The term breath-holding spells is actually a misnomer, as these are not self-induced, but
result from the immaturity of the autonomic system.
• The spells can be either Pallid or cyanotic type.
• Commonly seen in developmentally normal child occurring numerous times a day to one
episode a year.
3. Etiology
• A positive family history is present in 35% of children with spells suggesting some
genetic association.
• Studies suggest a maturational delay in myelination of brainstem to have a possible
role in etiology.
• Other studies reported to show altered selenium and antioxidant levels in children
with BHS.
4. Pathogenesis
• Both types of spells result from reflex changes that reduce cerebral blood flow.
• The first type is the pallid BHS, proposed to be due to parasympathetic system mediated
cardiac inhibition leading to bradycardia. The primary mechanism is due to increased vagal
tone leading to cerebral hypoperfusion.
• The second type is the cyanotic BHS, which does not occur during inspiration, but results
from prolonged expiratory apnea and intrapulmonary shunting.
• Anemia is believed to have strong association with the disorder. Regardless of the type of
spells, Iron deficiency anemia is known to prolong the duration of asystole during spells.
• Low levels of Hb results in reduced oxygen carrying capacity and prolonged cerebral anoxia.
5. Clinical Features
• Episodes start between the ages of 6 to 18 months.
• Attack frequency varies from many per day to only few irregular intervals and may increase
during the 2nd year of life.
• Typical Characteristic sequence consists of
1) A provocative stimulus
2) Apnea and Color change
3) Limpness followed by abnormal posturing
4) Stupor
6. Cyanotic BHS
• Most common type
• Usually occurs in response to anger, leading to vigorous cry, followed by apnea rapid
onset of cyanosis.
• May or may not be followed by loss of consciousness
• Abnormal posturing or repeated generalized clonic jerks.
• Usually regains consciousness within a minute, and resumes normal activities.
7. Pallid BHS
• Seen in about 25% of children.
• Usually occurs in response to fright and pain or an unexpected event.
• The child may grasp or cry, stops breathing, becomes hypotonic and loses
consciousness
• Clonic movements may occur, sometimes associated with prolonged asystole
• After regaining consciousness, pallid spells occasionally followed by sleep for several
hours.
8. Diagnosis
• Depends only on good and detailed clinical history, describing the entire
episodes as and when it occurred.
• It must also include precipitating event like emotional stimuli or trauma.
• Presence of urinary incontinence, uprolling of eyeballs, and deviation of
mouth are commonly seen with seizures, especially if not preceded by a cry.
• A complete physical examination including growth & development is
essential, especially cardiovascular examination for rhythm disturbances.
• No imaging or specific lab investigation is necessary to make the diagnosis.
9. Management
• Reassurance to parents regarding the benign nature of BHS is the mainstream of treatment.
• During the episode parents are requested to place the child in lateral recumbent position as
it shortens cerebral anoxia.
• Iron therapy must be initiated in all children with BHS with or without IDA.
• According to Cochrane review published in 2010, iron supplementation at 5mg/kg/day of
elemental iron for 16 weeks appears to reduce the frequency and severity of the spells.
• Atropine (0.01mg/kg twice or thrice a day) has been shown to be effective in pallid type.
• Use of Piracetam (40mg/kg/day) is proven to be safe and effective in severe BHS.
