{"id":4713,"date":"2023-02-26T09:40:40","date_gmt":"2023-02-26T09:40:40","guid":{"rendered":"https:\/\/neuropediatoolbox.org\/?p=4713"},"modified":"2026-06-22T20:07:15","modified_gmt":"2026-06-22T20:07:15","slug":"trastornos-del-desarrollo-de-la-via-visual-y-el-quiasma-optico","status":"publish","type":"post","link":"https:\/\/neuropediatoolkit.org\/de\/trastornos-del-desarrollo-de-la-via-visual-y-el-quiasma-optico\/","title":{"rendered":"St\u00f6rungen der Entwicklung der Sehbahn (und des Chiasma opticum)."},"content":{"rendered":"\n

El albinismo oculocut\u00e1neo es un trastorno gen\u00e9tico que condiciona no s\u00f3lo una retinopat\u00eda, sino otras alteraciones en el desarrollo embriol\u00f3gico de la via visual, y por lo tanto del neurodesarrollo. La principal consecuencia es una p\u00e9rdida de la estereopsia.<\/p>\n\n\n\n

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1.<\/div>
Herrera E, Garcia-Frigola C. Genetics and development of the optic chiasm. FBL [Internet]. 2008 Jan 1 [cited 2023 Feb 26];13(5):1646\u201353. Available from: https:\/\/www.imrpress.com\/journal\/FBL\/13\/5\/10.2741\/2788<\/a><\/div>\n <\/div>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n<\/figcaption><\/figure>\n\n\n\n

Existen otros trastornos del neurodesarrollo que tambi\u00e9n comparten caracter\u00edsticas con el albinismo oculocut\u00e1neo, como el s\u00edndrome de Prader-Willi, en el que en ocasiones, se afecta el gen OCA2 por microdelecci\u00f3n, por lo que adem\u00e1s de presentar hipopigmentaci\u00f3n, tienen alteraciones del enrutamiento de los axones a trav\u00e9s del nervio \u00f3ptico. <\/p>\n\n\n\n

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Spritz RA, Bailin T, Nicholls RD, Lee ST, Park SK, Mascari MJ, et al. Hypopigmentation in the Prader-Willi Syndrome Correlates With P Gene Deletion But Not With Haplotype of the Hemizygous P Allele. Am J Med Genet [Internet]. 1997 July 11 [cited 2023 Feb 26];71(1):57\u201362. Available from: https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC6067925\/<\/a><\/div>\n <\/div>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n<\/figcaption><\/figure>\n\n\n\n

Este tipo de trastornos de la via visual pueden ponerse de manifiesto neurofisiol\u00f3gicamente, a trav\u00e9s de la realizaci\u00f3n de PEV con estimulaci\u00f3n diferencial de cada ojo por separado, y registro dicot\u00f3mico en ambos l\u00f3bulos occipitales. <\/p>\n\n\n\n

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Creel DJ, Bendel CM, Wiesner GL, Wirtschafter JD, Arthur DC, King RA. Abnormalities of the central visual pathways in Prader-Willi syndrome associated with hypopigmentation. N Engl J Med [Internet]. 1986 June 19;314(25):1606\u20139. Available from: http:\/\/www.nejm.org\/doi\/abs\/10.1056\/NEJM198606193142503<\/a><\/div>\n <\/div>\n<\/div>\n\t\t\t\t<\/div>\n\t\t\t<\/div>\n\t\t<\/div>\n\t<\/div>\n\n<\/figcaption><\/figure>\n","protected":false},"excerpt":{"rendered":"

Okulokutaner Albinismus ist eine genetische St\u00f6rung, die nicht nur eine Retinopathie, sondern auch andere Ver\u00e4nderungen in der embryonalen Entwicklung der Sehbahn und damit der neurologischen Entwicklung verursacht. Die Hauptfolge ist ein Verlust der Stereopsis. Es gibt andere neurologische Entwicklungsst\u00f6rungen, die ebenfalls Merkmale mit dem okulokutanen Albinismus teilen, wie zum Beispiel ... <\/p>\n

Lesen Sie weiter \u201eEntwicklungsst\u00f6rungen des Sehwegs (und des Chiasma opticum).\u201c<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":4714,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_themeisle_gutenberg_block_has_review":false,"footnotes":""},"categories":[48,9,43],"tags":[],"class_list":["post-4713","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-especificamente-pediatricos","category-neurodesarrollo","category-trastornos-del-neurodesarrollo","entry"],"_links":{"self":[{"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/posts\/4713","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/comments?post=4713"}],"version-history":[{"count":3,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/posts\/4713\/revisions"}],"predecessor-version":[{"id":8429,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/posts\/4713\/revisions\/8429"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/media\/4714"}],"wp:attachment":[{"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/media?parent=4713"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/categories?post=4713"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/de\/wp-json\/wp\/v2\/tags?post=4713"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}