{"id":4523,"date":"2023-02-19T18:10:27","date_gmt":"2023-02-19T18:10:27","guid":{"rendered":"https:\/\/neuropediatoolbox.org\/?p=4523"},"modified":"2026-06-22T16:28:58","modified_gmt":"2026-06-22T16:28:58","slug":"trayectoria-de-desarrollo-de-la-mld-leucodistrofia-metacromatica","status":"publish","type":"post","link":"https:\/\/neuropediatoolkit.org\/fr\/trayectoria-de-desarrollo-de-la-mld-leucodistrofia-metacromatica\/","title":{"rendered":"Trajectoire de d\u00e9veloppement de la LMD (leucodystrophie m\u00e9tachromatique)."},"content":{"rendered":"<h2 class=\"wp-block-heading\">Leucodystrophie m\u00e9tachromatique (MLD)\u00a0: trajectoires de d\u00e9veloppement<\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">La leucodystrophie m\u00e9tachromatique est une maladie h\u00e9r\u00e9ditaire de stockage lysosomal autosomique r\u00e9cessive caract\u00e9ris\u00e9e par un d\u00e9ficit de l'enzyme arylsulfatase A (ARSA), provoquant l'accumulation de sulfatides et une d\u00e9my\u00e9linisation progressive du syst\u00e8me nerveux central et p\u00e9riph\u00e9rique.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\">Formes cliniques et trajectoire<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n    <li><strong>Nourrisson tardif (typique)\u00a0:<\/strong> Elle appara\u00eet entre la premi\u00e8re et la deuxi\u00e8me ann\u00e9e de vie. Les nourrissons perdent la capacit\u00e9 de marche acquise, pr\u00e9sentent une r\u00e9gression motrice s\u00e9v\u00e8re, une quadripar\u00e9sie spastique progressive et la c\u00e9cit\u00e9.<\/li>\n    <li><strong>Jeunes et adultes\u00a0:<\/strong> Progression plus lente, initialement caract\u00e9ris\u00e9e par des difficult\u00e9s d'apprentissage, des troubles du comportement, une ataxie et un d\u00e9ficit moteur global ult\u00e9rieur.<\/li>\n<\/ul>","protected":false},"excerpt":{"rendered":"<p>Leucodystrophie m\u00e9tachromatique (MLD)\u00a0: trajectoires de d\u00e9veloppement La leucodystrophie m\u00e9tachromatique est une maladie de stockage lysosomal h\u00e9r\u00e9ditaire autosomique r\u00e9cessive caract\u00e9ris\u00e9e par un d\u00e9ficit de l'enzyme arylsulfatase A (ARSA), provoquant l'accumulation de sulfatides et une d\u00e9my\u00e9linisation progressive du syst\u00e8me nerveux central et p\u00e9riph\u00e9rique. Formes cliniques et trajectoire tardive de l'enfance (typique)\u00a0: elle se manifeste entre le premier\u2026 <\/p>\n<p class=\"link-more\"><a href=\"https:\/\/neuropediatoolkit.org\/fr\/trayectoria-de-desarrollo-de-la-mld-leucodistrofia-metacromatica\/\" class=\"more-link\">Continuer la lecture<span class=\"screen-reader-text\"> \"Trajectoire d\u00e9veloppementale de la MLD (leucodystrophie m\u00e9tachromatique).\"<\/span><\/a><\/p>","protected":false},"author":1,"featured_media":4525,"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-4523","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\/4523","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=4523"}],"version-history":[{"count":2,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts\/4523\/revisions"}],"predecessor-version":[{"id":8292,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/posts\/4523\/revisions\/8292"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/media\/4525"}],"wp:attachment":[{"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/media?parent=4523"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/categories?post=4523"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/neuropediatoolkit.org\/fr\/wp-json\/wp\/v2\/tags?post=4523"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}