{"id":4520,"date":"2023-02-19T18:08:37","date_gmt":"2023-02-19T18:08:37","guid":{"rendered":"https:\/\/neuropediatoolbox.org\/?p=4520"},"modified":"2026-06-22T16:28:59","modified_gmt":"2026-06-22T16:28:59","slug":"trayectorias-de-desarrollo-de-las-cln","status":"publish","type":"post","link":"https:\/\/neuropediatoolkit.org\/fr\/trayectorias-de-desarrollo-de-las-cln\/","title":{"rendered":"Trajecto de d\u00e9veloppement des LNC."},"content":{"rendered":"<h2 class=\"wp-block-heading\">Lipofuscinose neuronale c\u00e9ro\u00efdienne (CLN)\u00a0: trajectoires de d\u00e9veloppement<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">La c\u00e9ro\u00efde lipofuscinose neuronale (CLN), commun\u00e9ment appel\u00e9e maladie de Batten, regroupe un groupe de maladies neurod\u00e9g\u00e9n\u00e9ratives rares \u00e0 base lysosomale caract\u00e9ris\u00e9es par l'accumulation intralysosomale de mat\u00e9riel fluorescent de c\u00e9ro\u00efde lipofuscine.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Chemin commun des sympt\u00f4mes<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n    <li>Perte progressive et rapide de l\u2019acuit\u00e9 visuelle conduisant \u00e0 la c\u00e9cit\u00e9 (souvent le premier signe).<\/li>\n    <li>Crises d'\u00e9pilepsie myocloniques et focales r\u00e9fractaires au traitement pharmacologique.<\/li>\n    <li>R\u00e9gression progressive des \u00e9tapes cognitives (d\u00e9mence) et motrices.<\/li>\n    <li>D\u00e9ficience motrice g\u00e9n\u00e9ralis\u00e9e avec spasticit\u00e9 profonde et d\u00e9c\u00e8s pr\u00e9matur\u00e9.<\/li>\n<\/ul>","protected":false},"excerpt":{"rendered":"<p>Lipofuscinose neuronale c\u00e9ro\u00efde (CLN) : trajectoires de d\u00e9veloppement La lipofuscinose neuronale c\u00e9ro\u00efde (CLN), commun\u00e9ment appel\u00e9e maladie de Batten, regroupe un ensemble de maladies neurod\u00e9g\u00e9n\u00e9ratives rares \u00e0 base lysosomale caract\u00e9ris\u00e9es par l'accumulation intralysosomale de mat\u00e9riel fluorescent de lipofuscine c\u00e9ro\u00efde. Cheminement courant des sympt\u00f4mes Perte progressive et rapide de l'acuit\u00e9 visuelle conduisant \u00e0 la c\u00e9cit\u00e9 (\u00e0... <\/p>\n<p class=\"link-more\"><a href=\"https:\/\/neuropediatoolkit.org\/fr\/trayectorias-de-desarrollo-de-las-cln\/\" class=\"more-link\">Continuer la lecture<span class=\"screen-reader-text\"> \u00abTrajectoires de d\u00e9veloppement des CLN.\u00bb<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":4535,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_themeisle_gutenberg_block_has_review":false,"footnotes":""},"categories":[49],"tags":[],"class_list":["post-4520","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-neurodegenerativas","entry"],"_links":{"self":[{"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts\/4520","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/comments?post=4520"}],"version-history":[{"count":2,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts\/4520\/revisions"}],"predecessor-version":[{"id":8293,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts\/4520\/revisions\/8293"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/media\/4535"}],"wp:attachment":[{"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/media?parent=4520"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/categories?post=4520"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/tags?post=4520"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}