PORENCEPHALY

Abstract

A child who died at the age of 11 mos. was said to have been normal until the age of 4 mos., when it was noted that the back became stiff and the head was thrown back. On examination the child was found to be in marked opisthotonos. The upper limbs were fixed in moderate hyperextension with strong extension at the elbow and wrist joints and with pronation of the hands. The lower extremites were in strong forced adduction and extension. The feet were dorsiflexed and the toes were widely separated. At autopsy there was found to be a dysgenesis of the neo-cerebrum and internal hydrocephalus which gave rise to distortion of the thalamus and brain stem. The hydrocephalus involved only the lateral ventricles. The 3d ventricle was of normal dimensions and the aqueduct was tortuous due to compression and flattening of the tectum. It was believed that a primary neuronal dysplastic process was responsible for the abnormality.