The syndrome of absence of the septum pellucidum with porencephalies and other developmental defects

Abstract

A patient with absence of the septum peilucidum, porencephalies and heterotopies of grey matter is reported. A spastic tetraparesis, nistagmus, ocular paralysis and mental retardation were present at clinical examination. The clinical and radiological picture findings were compatible with a syndrome of absence of the septum peilucidum with porencephalies. The most striking aspects of this picture are commented.