Polycystic brain (cerebrum polycystica vera) associated with ectodermal dysplasia: A new neurocutaneous syndrome
- 64 Downloads
This 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.
KeywordsRetinal Pigment Epithelium Retinal Pigment Pigment Epithelium Unique Case Retinal Cell
Unable to display preview. Download preview PDF.
- 1.Atlas SW (1991) Magnetic resonance imaging of the brain and spine. Raven Press, New York, pp 569–573Google Scholar
- 2.Barkovich AJ (1990) Pediatric neuroimaging. Raven Press, New York, pp 61–64Google Scholar
- 3.Wolpert SM, Barnes PD (1992) MRI in pediatric neuroradiology. Mosby Year Book, St Louis, pp 178–179Google Scholar
- 4.Poznanski AK (1984) The hand in radiologic diagnosis, vol 1, 2nd edn. Saunders, Philadelphia, pp 415–416Google Scholar
- 5.Williams PL, Warwick R, Dyson M, Bannister LH (1989) Gray's anatomy. 37th edn. Churchill Livingstone, Edinburgh, pp 136–138Google Scholar
- 6.Barnes PD, Korf BR (1992) Neurocutaneous syndromes. In: Wolpert SM, Barnes PD (eds) MRI in pediatric neuroradiology. Mosby Year Book, St Louis, pp 299–327Google Scholar
- 7.Walsh PC, Gittes RF, Perlmutter AD, Stamey TA (1986) Campbell's urology, vol 2, 5th edn. Saunders, Philadelphia, pp 1767–1774Google Scholar