Polycystic Brain (Cerebrum Polycystica-Vera) Associated With Ectodermal Dysplasia - a New Neurocutaneous Syndrome

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dc.contributor.authorSener, Rn
dc.date.accessioned2019-10-27T11:35:33Z
dc.date.available2019-10-27T11:35:33Z
dc.date.issued1994
dc.departmentEge Üniversitesien_US
dc.description.abstractThis 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.en_US
dc.identifier.doi10.1007/BF02020167en_US
dc.identifier.endpage118en_US
dc.identifier.issn0301-0449
dc.identifier.issue2en_US
dc.identifier.pmid8078711en_US
dc.identifier.startpage116en_US
dc.identifier.urihttps://doi.org/10.1007/BF02020167
dc.identifier.urihttps://hdl.handle.net/11454/33736
dc.identifier.volume24en_US
dc.identifier.wosWOS:A1994NJ37200010en_US
dc.identifier.wosqualityN/Aen_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherSpringer Verlagen_US
dc.relation.ispartofPediatric Radiologyen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/closedAccessen_US
dc.titlePolycystic Brain (Cerebrum Polycystica-Vera) Associated With Ectodermal Dysplasia - a New Neurocutaneous Syndromeen_US
dc.typeArticleen_US

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