Abstracts

To determine the clinical characteristics of startle epilepsy characterized by reflex partial seizure of sensorimotor cortex Eight patients with startle epilepsy were evaluated for clinical seizure history, neurologic examination, MRI, routine EEG, video-EEG monitoring, and ictal SPECT Etiologies were perinatal injury(4 patients), kernicterus following hemorrhage during infancy(1 patient), near-drowning at age 4(1), and unknown(2). Age of sezure onset varied from several months to 16 years. All patients had spontaneous as well as reflex seizures. The effective triggers were several sensory modalities, typically proprioceptive and auditory. The stimuli should be sudden and unexpected. Their seizures usually occurred daily and were medically intractable. Neurologic examination was abnormal in 6 patients. Five had mental retardation, and 4 motor deficit. MRI was abnormal in 6 patients, generally large encephalomalatic changes involving cortical and subcortical structures. Five had bilateral lesions. The semiology was characterized by unilateral or generalized tonic posture. Scalp interictal and ictal EEGs had limited value to lateralize the epileptogenic focus. Intracranial EEG in one patient who underwent surgery showed ictal onset on lateral sensorimotor cortex. Startle epilepsy is a rare but distinctive epileptic syndrome characterized by reflex seizure triggered by unexpected, sudden sensory stimuli. Some clinical findings including etiologic events(kernicterus following hemorrhage, near-drowning), high incidence of bilateral lesions, ictal onset on lateral sensorimotor cortex without involvement of medial cortex, and ictal SPECT findings are not reported or different from previous studies.