Six neurologically normal infants, aged 6-21 months, with attacks resembling benign myoclonic epilepsy of infancy but occurring as reflex responses to auditory and tactile stimuli are reported from Bambino Gesu Children’s Hospital, Rome, Italy. An excessive startle response caused symmetric jumping of limbs, arms more than legs. Spontaneous attacks developed in 4, particularly in sleep. A family history of epilepsy or febrile convulsions was elicited in 5. Remissions occurred in 4-14 months, spontaneously or in response to valproate. EEGs showed short generalized spike- or polyspike-and-wave discharges. [1]
COMMENT. In addition to the reflex nature of these attacks, the abnormal EEG appears to distinguish this syndrome from benign myoclonic epilepsy in infancy. Therapy with anticonvulsants seemed justified when attacks occurred spontaneously and frequently, from 5 to 20 times daily.