Abstract
We are reporting a case of rare reflex epilepsy—“startle” epilepsy effectively treated
with hemispherotomy. A 6-year-old boy, a child of nonconsanguineous parentage with
infantile hemiparesis presented to us with recurrent right focal seizures along with
secondary generalization provoked by sudden and unexpected auditory stimuli for the
last 1 year. The boy had a minimum of four episodes per day despite maximal medical
management with multiple antiepileptic drugs. Magnetic resonance imaging of the brain
showed severe atrophy of the whole of the left hemisphere. Interictal electroencelphographic
(EEG) data showed predominantly left central epileptiform discharges. Long-term video-EEG
monitoring record showed seizures of the left hemispheric origin. He underwent a left
functional hemispherotomy and has been totally seizure free in the 12-month follow-up
period with an improved quality of life both for him and for his caregivers. We feel
that surgical management should be considered in startle epilepsy with infantile hemiparesis
when seizures are refractory to the optimal medical treatment.
Keywords
startle epilepsy - infantile hemiparesis - hemispherotomy
