Fixation-off sensitivity in an adult with symptomatic occipital epilepsy

An adult patient had EEG occipital spike fixation-off sensitivity and spontaneous occipital seizures due to perinatal asphyxia with bioccipital hemorrhage. EEG abnormalities consisted of repetitive posterior spikes that occurred when the eyes were closed and other conditions of fixation-off. Magnetic resonance imaging (MRI) documented severe posterior lesions with wallerian degeneration of the optic radiation. Electron capture detector single-photon emission computed tomography (ECD-SPECT) during the state of fixation-off showed regional hyperperfusion in the right posterior regions. We suggest that partial denervation of the primary visual cortex in this patient resulted in an increased cortical excitability and that the inhibitory effect of central vision and fixation leads to a suppression of spontaneous epileptic activity. This unusual symptomatic case may serve as a model for the pathophysiology in the more often reported cases of idiopathic benign epilepsy syndrome of childhood.