Complete heart block in nonfamilial hyperekplexia

Pediatr Neurol. 1995 Feb;12(2):149-51. doi: 10.1016/0887-8994(94)00111-e.

Abstract

An infant with nonfamilial hyperekplexia had multiple episodes of tonic spasms that mimicked tonic seizures. They were accompanied by complete heart block and apnea. These episodes did not correlate with electroencephalographic epileptiform changes and were partially responsive to clonazepam, valproic acid, and cardiac pacemaker. Sudden death in hyperekplexia may be related to complete heart block and apnea during seizurelike episodes.

Publication types

  • Case Reports

MeSH terms

  • Apnea / diagnosis
  • Apnea / genetics
  • Apnea / physiopathology
  • Atrioventricular Node / physiopathology
  • Cerebral Cortex / physiopathology
  • Child, Preschool
  • Diagnosis, Differential
  • Electrocardiography, Ambulatory
  • Epilepsy, Generalized / diagnosis
  • Epilepsy, Generalized / genetics
  • Epilepsy, Generalized / physiopathology*
  • Female
  • Follow-Up Studies
  • Heart Block / diagnosis
  • Heart Block / genetics
  • Heart Block / physiopathology*
  • Humans
  • Infant
  • Infant, Newborn
  • Muscle Hypertonia / diagnosis
  • Muscle Hypertonia / genetics
  • Muscle Hypertonia / physiopathology
  • Neurologic Examination
  • Reflex, Abnormal / genetics
  • Reflex, Abnormal / physiology*
  • Reflex, Startle / genetics
  • Reflex, Startle / physiology*