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Related Experiment Videos

Complete heart block in nonfamilial hyperekplexia

G N McAbee1, S K Kadakia, K C Sisley

  • 1Department of Neurology, Nassau County Medical Center, East Meadow, NY 11554, USA.

Pediatric Neurology
|February 1, 1995
PubMed
Summary

Nonfamilial hyperekplexia in an infant caused seizure-like episodes with heart block and apnea. These events, unresponsive to anti-epileptic drugs, suggest a link between hyperekplexia, cardiac issues, and sudden infant death.

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Area of Science:

  • Neurology
  • Pediatrics
  • Cardiology

Background:

  • Hyperekplexia is a rare neurological disorder characterized by exaggerated startle responses.
  • Nonfamilial cases present unique diagnostic challenges, particularly in differentiating from other conditions like epilepsy.

Observation:

  • An infant experienced recurrent tonic spasms mimicking seizures.
  • These episodes were associated with complete heart block and apnea.
  • Electroencephalography showed no epileptiform activity during these events.

Findings:

  • The seizure-like episodes were partially responsive to clonazepam, valproic acid, and a cardiac pacemaker.
  • The lack of correlation with EEG epileptiform changes suggests a non-epileptic origin.
  • Complete heart block and apnea during these episodes were key features.

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Implications:

  • Sudden death in hyperekplexia may be linked to complete heart block and apnea during seizure-like episodes.
  • This case highlights the importance of considering non-epileptic events in infants with hyperekplexia.
  • Comprehensive monitoring, including cardiac and respiratory, is crucial for managing infants with severe hyperekplexia.