Immunocytochemical studies on a fetal brain of tuberous sclerosis

A female stillborn baby was delivered at 36 weeks' gestation. There were rhabdomyomas in the heart, multi-cystic kidneys, and tubers in the brain. Neuropathologic examination of the brain tissue revealed typical giant cells in the sub-ependymal nodules, cerebral white matter, and cortex. On immunocytochemical staining, the giant cells stained positively with antisera to glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), and neuron-specific enolase (NSE). The giant cells as well as adjacent glial cells with small eccentric nuclei contained both MBP and GFAP. Both cell types combined immunocytochemical characteristics of astrocytes and oligodendrocytes. There were also intermediate-sized cells found among giant cells and germ cells in subependymal nodules. In white matter nodules, similar intermediate-sized cells were found among giant cells and glial cells with small eccentric nuclei. Therefore, giant cells as well as glial cells with small eccentric nuclei may be multi-potential and may result from abnormal patterns of maturation.