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Septo-optic Dysplasia Plus Syndrome

Septo-optic dysplasia plus is a rare congenital syndrome characterized by the classic triad of optic nerve hypoplasia, hypothalamic-hypophyseal dysfunction, and midline abnormalities, with associated malformations of cortical development. Clinical manifestations include optic nerve disease, epilepsy...

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Detalles Bibliográficos
Autores principales: Gutierrez-Castillo, Alejandro, Jimenez-Ruiz, Amado, Chavez-Castillo, Melissa, Ruiz-Sandoval, José Luis
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384050/
https://www.ncbi.nlm.nih.gov/pubmed/30800538
http://dx.doi.org/10.7759/cureus.3727
Descripción
Sumario:Septo-optic dysplasia plus is a rare congenital syndrome characterized by the classic triad of optic nerve hypoplasia, hypothalamic-hypophyseal dysfunction, and midline abnormalities, with associated malformations of cortical development. Clinical manifestations include optic nerve disease, epilepsy, intellectual delay, and endocrine dysfunction. We present the case of an 18-year-old man with a history of seizures, growth hormone deficiency, and optic nerve disease that was diagnosed with septo-optic dysplasia plus syndrome with characteristic imaging findings.