Is a seizure associated with breath-holding spells considered a severe manifestation?

Seizures Associated with Breath-Holding Spells: Severity Assessment

Seizures associated with breath-holding spells are considered a severe manifestation that may require more aggressive management approaches, particularly when associated with documented asystole or prolonged loss of consciousness. 1, 2

Classification and Understanding of Breath-Holding Spells with Seizures

• Breath-holding spells with seizure-like activity are classified as "complex breath-holding spells" and represent a more severe form of the condition compared to simple breath-holding spells without syncope 1
• Pallid breath-holding spells with seizures represent cardioinhibitory vasovagal syncope in small children, where respiration is not involved in the pathophysiology, but rather a cardiac reflex causing asystole 1
• Cyanotic breath-holding spells represent a unique form of transient loss of consciousness in small children involving involuntary expiratory apnea followed by secondary circulatory events 1
• The presence of seizure-like movements during breath-holding spells is often due to cerebral hypoperfusion, not epilepsy, with studies showing 64% of children with syncope and convulsions exhibiting cardiac asystole with pauses >3 seconds 1

Severity Assessment and Management Implications

• Breath-holding spells with seizure activity require more aggressive evaluation and management compared to simple spells without syncope 2, 3
• The American College of Cardiology/American Heart Association guidelines consider breath-holding spells with seizures severe enough to warrant consideration of cardiac pacing in cases with documented asystole 1
• In children with episodes of vasovagal syncope that mimic epileptic seizures, upright tilt-table testing combined with graded isoproterenol infusion has identified cardiac causes in 42% to 67% of patients previously thought to have primary seizure disorders 1
• A combined cardiology and neurology evaluation is warranted in patients with breath-holding spells and seizure-like activity to rule out both cardiac and neurological causes 1

Management Approaches for Severe Cases

• For severe cases with documented asystole, cardiac pacing may be considered, with studies showing 86% of infants and toddlers experiencing either complete resolution or significant reduction in syncopal events with pacing 1, 2
• Midodrine therapy should be considered for persistent, severe cases with seizures, as it has shown significant reduction in recurrence rates from 80% to 22% 1, 2
• Iron supplementation should be provided for children with iron deficiency, as it has been shown to reduce frequency and severity of spells, even in children without anemia 2, 4
• Beta-blockers are not recommended for pediatric patients with vasovagal syncope and seizures, as studies show they may actually increase recurrence rates 1, 3
• For refractory cases with seizures, alternative treatments such as piracetam have shown effectiveness in some studies, with complete resolution in 81% of children with severe breath-holding spells 5

Monitoring and Follow-up

• Children with breath-holding spells and seizures require closer monitoring and follow-up compared to those with simple spells 2
• Emergency medical services should be activated for seizures lasting >5 minutes during breath-holding episodes 2
• Parents should be instructed to place the child in the recovery position during episodes to prevent injury and not to restrain the child or put anything in the mouth 2
• Regular follow-up is essential to monitor response to treatment and adjust management strategies as needed 2, 3