GLOBAL CEREBRAL DYSPLASIA DUE TO DYSPLASIA AND HYPERPLASIA OF PERIVENTRICULAR GERMINAL CELLS

Complete obliteration of the cerebral ventricular cavities has not bee n previously described as an entity among central nervous system malfo rmations. Markedly disorganized maldevelopment of the deep cerebral nu clei and moderately disorganized maldevelopment of the cerebral cortex were observed in the brains of two Cases, a 3-year-old female and a m ale newborn infant. One showed complete and the other partial oblitera tion of the lateral and third ventricles. Both showed severe maldevelo pment of the basal ganglia, which were represented by a large central mass consisting of randomly distributed multiple nodules of gray matte r without any discernible differentiation into lenticulostriate nuclei , thalamus, or hypothalamus. Minor malformations involved the cerebral cortex, cerebellum, and mesencephalon to varying degrees. Each of the above two cases was thought to be unique and different until the brai n of a fetus of 155 days' gestation was studied. This fetal brain show ed exuberant and disorganized periventricular proliferation and migrat ion of germinal matrix cells, with obliteration of the lateral and thi rd ventricles. It was concluded that hyperplasia and disorganized migr ation of the periventricular germinal matrix early in gestation of wha tever cause itself can result in severe maldevelopment of the deep cer ebral nuclei and obliteration of the ventricular cavities.