Twenty-six cases of startle epileptic seizures (SES) were studied. The
mean age at seizure onset was 5.1 years, with six patients starting i
mmediately after birth. Severe brain damage was observed in 17 patient
s (65%). Epilepsy was severe and intractable in 21 patients (80.7%). I
maging studies (magnetic resonance imaging and computed tomography) we
re abnormal in 17 cases, although no single pattern of lesion was foun
d. Most of these patients had large brain lesions, either unilateral (
n = 7) or bilateral (n = 10). The corpus callosum was affected in five
cases (19.2%). We conclude that SES is a heterogeneous entity that co
uld be associated with different types of seizures and epilepsies. SES
is more frequently associated with severe brain damage and intractabl
e epilepsy. No clear-cut imaging pattern was found in association with
SES.