An adult patient had EEG occipital spike fixation off sensitivity and spont
aneous occipital seizures due to perinatal asphyxia with bioccipital hemorr
hage. EEG abnormalities consisted of repetitive posterior spikes that occur
red when the eyes were closed and other conditions of fixation-off. Magneti
c resonance imaging (MRI) documented severe posterior lesions with walleria
n degeneration of the optic radiation. Electron capture detector single-pho
ton emission computed tomography (ECD-SPECT) during the state of fixation-o
ff showed regional hyperperfusion in the right posterior regions. We sugges
t that partial denervation of the primary visual cortex in this patient res
ulted in an increased cortical excitability and that the inhibitory effect
of central vision and fixation leads to a suppression of spontaneous epilep
tic activity. This unusual symptomatic case may serve as a-model for the pa
thophysiology in the more often reported cases of idiopathic benign epileps
y syndrome of childhood.