Child Neurology: Neurophysiologic and Anatomical Correlates in Startle Epilepsy

Startle epilepsy, characterized by startle-provoked epileptic seizures, was historically recognized as one of the reflex epilepsies but currently lacks classification as a specific epileptic syndrome because of insufficient characterization. This study presents an institutional experience and review of relevant literature focusing on the neurophysiologic and anatomical aspects of startle epilepsy. We describe a pediatric patient with an underlying structural etiology of left frontal encephalomalacia who continued to experience disabling seizures despite multiple antiseizure medications and previous palliative surgery. A comprehensive presurgical evaluation using SEEG led to the resection of the left supplementary motor area and adjacent middle cingulate cortex, resulting in successful seizure remission. A literature review on the surgical treatment of startle epilepsy revealed consistent reports of successful seizure remission through neocortical resection of the supplementary motor area and/or cingulate region. This case study and literature review highlights startle epilepsy as a distinct form of epilepsy with identifiable neurophysiologic and anatomical characteristics. Our observations emphasize the potential of resective surgery as a viable treatment option for startle epilepsy and underscore the importance of neurophysiologic monitoring in guiding surgical interventions.