Electrophysiological Study on a Case with Schizencephaly

Abstract

A 11-month-old boy was a product of the first pregnancy of a 40-year-old mother who had used a betamethasone, bronchodilators and antihistaminics during pregnancy. His birth weight was 2, 280 g. There was not birth asphyxia. He smiled and controlled the head at 3 months of age. Internal strabismus and visual impairment were noticed at 4 months. He creeped on the prone position at 8 months. There were no epileptic fits.
On neurological examination at 11 months, there were abnormal eyeball movements and spastic paraplegia. There were no paresis of upper extremities. He could vocalize sound, but no intelligible words. Parachute reflex was not present.
Plain CT showed symmetrical, large and well-defined low density areas in the parieto-temporal regions which appeared to communicate with subarachnoid space at the occipital horn. Defect of septum pellucidum was also demonstrated.
EEG showed localized irregular polyspike and wave complexes independently in the bilateral parietooccipital areas. Visual evoked potential was not elicited. Auditory brain stem responses showed normal latency of wave V. Short latency somatosensory evoked potentials showed normal latency of each waves except relatively low amplitude of N1 compared with normal controls.
These electrophysiological findings suggests that clefts transverse the optic radiations and involve a part of somatosensory and somatomotor areas, but the sensory function in the brain stem and thalamus are normal.