We report a case of non-familial hyperekplexia which characteristicall
y developed apnea and feeding difficulties in the neonatal period, The
abnormal startle response was evident from the second week of life on
wards, 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 o
n EMG examination, suggesting a possible cortical neuronal hyperexcita
bility origin for the abnormal startle response observed in hyperekple
xia.