Sener R.N.2019-10-272019-10-2719940301-0449https://doi.org/10.1007/BF02020167https://hdl.handle.net/11454/28232This paper presents a unique case of true polycystic brain in which multiple cysts of curvilinear, round, oval, or layered configuration occurred. These apparently represented extremely dilated Virchow-Robin spaces: the perivascular spaces lined by ependymal/leptomeningeal cells. Irregular retinal pigment epithelium was also evident. In addition, the patient showed ectodermal dysplasia manifesting as thin hair, dystrophic nails, and dental abnormalities. A common ectodermal origin for the brain cysts and the ectodermal changes is proposed, as it is known that the central nervous system (including the ependymal/leptomeningeal cells and the retinal cells), the epidermis (including hair and nails), and the enamel of the teeth have the same origin - the embryonic ectoderm. This association appears to be a new, distinct neurocutaneous syndrome. © 1994 Springer-Verlag.en10.1007/BF02020167info:eu-repo/semantics/closedAccessArticle2421161188078711N/A