Hyperekplexia, a cause of neonatal apnea: a case report

Brain Dev. 1995 Mar-Apr;17(2):114-6. doi: 10.1016/0387-7604(94)00113-c.


We report a case of non-familial hyperekplexia which characteristically developed apnea and feeding difficulties in the neonatal period. The abnormal startle response was evident from the second week of life onwards. The infant showed a marked improvement of the startle response and muscle hypertonia with clonazepam. Clobazam was also tried with no apparent response. A prominent long latency C response was observed on EMG examination, suggesting a possible cortical neuronal hyperexcitability origin for the abnormal startle response observed in hyperekplexia.

Publication types

  • Case Reports

MeSH terms

  • Anti-Anxiety Agents*
  • Apnea / etiology*
  • Benzodiazepines*
  • Benzodiazepinones / therapeutic use
  • Clobazam
  • Clonazepam / therapeutic use
  • Electromyography
  • Female
  • Humans
  • Infant, Newborn
  • Movement Disorders / diagnosis
  • Movement Disorders / physiopathology*
  • Reflex
  • Reflex, Startle*


  • Anti-Anxiety Agents
  • Benzodiazepinones
  • Benzodiazepines
  • Clobazam
  • Clonazepam