Abstract
There are many types of congenital malformation of the brain, and its diagnosis was made by postmortem examinations. The recent advent of computed tomography (CT scan), however, has made it possible to diagnose brain anomalies in living subjects. Pachygyria is a rare developmental malformation of the brain characterized by a few broad gyri and shallow sulci. This report presents a patient with pachygyria who had seizures before one year of age, microcephaly, micrognathia, carp mouth, hypotonia, motor retardation, and dysrhythmic EEG pattern with spike discharges. She was treated with ACTH-Z successfully, which could control the tonic seizures and EEG findings completely. In spite of the detailed descriptions of the clinical and neuropathological features of lissencephaly syndrome, there have been only few reports concerning EEG findings. In this case EEG and some evoked potentials were studied. Low amplitude of wave V in ABR and poor reproducibility in the short latency somatosensory evoked potential were shown, and the blink reflex elicited by photic stimulation indicated prolonged wave latency. It seems that these findings show existence of midbrain and thalamus dysfunctions.
