{"id":74243,"date":"2021-11-23T16:30:17","date_gmt":"2021-11-23T16:30:17","guid":{"rendered":"https:\/\/www.atlasrleye.com\/manifestations-oculaires-de-la-ceroide-lipofuscinose-neuronale\/"},"modified":"2025-07-15T00:59:01","modified_gmt":"2025-07-15T00:59:01","slug":"manifestations-oculaires-de-la-ceroide-lipofuscinose-neuronale","status":"publish","type":"page","link":"https:\/\/www.atlasrleye.com\/fr\/manifestations-oculaires-de-la-ceroide-lipofuscinose-neuronale\/","title":{"rendered":"MANIFESTATIONS OCULAIRES DE LA C\u00c9RO\u00cfDE-LIPOFUSCINOSE NEURONALE"},"content":{"rendered":"<div class=\"wpb-content-wrapper\"><p>[vc_row][vc_column][vc_custom_heading text=\u00a0\u00bbSYNDROMES ET DYSTROPHIES R\u00c9TINIENNES\u00a0\u00bb font_container=\u00a0\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000&Prime; use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Ffr%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00a0\u00bb][vc_text_separator title=\u00a0\u00bb\u00a0\u00bb i_icon_fontawesome=\u00a0\u00bbfas fa-eye\u00a0\u00bb i_color=\u00a0\u00bbcustom\u00a0\u00bb i_size=\u00a0\u00bblg\u00a0\u00bb color=\u00a0\u00bbcustom\u00a0\u00bb border_width=\u00a0\u00bb8&Prime; css=\u00a0\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00a0\u00bb add_icon=\u00a0\u00bbtrue\u00a0\u00bb accent_color=\u00a0\u00bb#bababa\u00a0\u00bb i_custom_color=\u00a0\u00bb#bababa\u00a0\u00bb][vc_custom_heading source=\u00a0\u00bbpost_title\u00a0\u00bb font_container=\u00a0\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52&Prime; use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb]<div class=\"ult-spacer spacer-6a03dc09685c8\" data-id=\"6a03dc09685c8\" data-height=\"30\" data-height-mobile=\"0\" data-height-tab=\"\" data-height-tab-portrait=\"\" data-height-mobile-landscape=\"\" style=\"clear:both;display:block;\"><\/div>[\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb3\/4&Prime;][vc_column_text]<\/p>\n<div class=\"w-100 middle-subtext text-justify hidden-text\">\n<p style=\"text-align: justify; font-size: 16px;\">La C\u00e9roide-Lipofuscinose Neuronale (NCL) est une maladie neurod\u00e9g\u00e9n\u00e9rative caus\u00e9e par une alt\u00e9ration du m\u00e9tabolisme des sphingolipides, provoquant une accumulation grave et progressive de lipofuscine dans les cellules du cerveau et d&rsquo;autres tissus. Les lipofuscinoses sont h\u00e9rit\u00e9s par un mode autosomique r\u00e9cessif.<\/p>\n<p style=\"text-align: justify; font-size: 16px;\">Il existe trois types de NLC: adulte (maladie de Kuf), juv\u00e9nile (maladie de Batten) et infantile tardive (maladie de Jansky-Bielschowsky). Les formes de c\u00e9roide-lipofuscinose chez l\u2019enfance sont fatales et les manifestations de la maladie comprennent des saisies, perte de la vision, d&rsquo;ataxie, perte de coordination motrice et d\u00e9t\u00e9rioration mentale.<\/p>\n<p style=\"text-align: justify; font-size: 16px;\">La NCL, initialement, entra\u00eene \u00e0 une perte de vision r\u00e9sultant d&rsquo;une r\u00e9tinopathie pigmentaire qui progresse rapidement et, plus tard, d\u2019une atrophie optique. Dans les formes pr\u00e9coces, cause c\u00e9cit\u00e9 environ de 2-3 ans.<\/p>\n<p style=\"font-size: 16px;\">Diagnostic diff\u00e9rentiel<\/p>\n<ul>\n<li style=\"font-size: 16px;\">Maladie de Canavan<\/li>\n<li style=\"font-size: 16px;\">Maladie de Krabb\u00e9<\/li>\n<li style=\"font-size: 16px;\">Syndrome de Leigh<\/li>\n<li style=\"font-size: 16px;\">Leucodystrophie m\u00e9tachromatique<\/li>\n<li style=\"font-size: 16px;\">Maladie de Niemann-Pick<\/li>\n<li style=\"font-size: 16px;\">Syndrome de Retts<\/li>\n<li style=\"font-size: 16px;\">Maladie de Tay-Sachs<\/li>\n<\/ul>\n<\/div>\n<p>[\/vc_column_text][\/vc_column][vc_column width=\u00a0\u00bb1\/4&Prime;][vc_images_carousel images=\u00a0\u00bb21719,21718&Prime; img_size=\u00a0\u00bbmedium\u00a0\u00bb speed=\u00a0\u00bb2000&Prime; autoplay=\u00a0\u00bbyes\u00a0\u00bb hide_pagination_control=\u00a0\u00bbyes\u00a0\u00bb wrap=\u00a0\u00bbyes\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1752540756233{margin-bottom: 10px !important;}\u00a0\u00bb]<div class=\"stats-block stats-left  \"><div class=\"aio-icon-left\"><div class=\"ult-just-icon-wrapper  \"><div class=\"align-icon\" style=\"text-align:center;\">\n<div class=\"aio-icon square \"  style=\"color:#333333;background:#ffffff;font-size:32px;display:inline-block;\">\n\t<i class=\"Defaults-eye\"><\/i>\n<\/div><\/div><\/div><\/div><div class=\"stats-desc\" id=\"counter-responsv-wrap-4107\"><div id=\"counter_17465062516a03dc0968a2a\" data-id=\"counter_17465062516a03dc0968a2a\"  data-ultimate-target='#counter-responsv-wrap-4107 .stats-number'  data-responsive-json-new='{\"font-size\":\"\",\"line-height\":\"\"}'  class=\"stats-number ult-responsive\" style=\"font-size:28px;  \" data-speed=\"3\" data-counter-value=\"1\" data-separator=\",\" data-decimal=\".\">0<\/div><div id=\"title-counter_17465062516a03dc0968a2a\"  data-ultimate-target='#counter-responsv-wrap-4107 .stats-text'  data-responsive-json-new='{\"font-size\":\"desktop:16px;\",\"line-height\":\"\"}'  class=\"stats-text ult-responsive\" style=\"font-size:px;  font-weight:bold;\">Cas Cliniques<\/div><\/div><\/div>[\/vc_column][\/vc_row][vc_row][vc_column][vc_column_text css=\u00a0\u00bb.vc_custom_1752540766064{margin-bottom: 10px !important;}\u00a0\u00bb]<\/p>\n<h1><strong><span style=\"color: #5f1b52;\">Cas Cliniques<\/span><\/strong><\/h1>\n<p>[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb1\/4&Prime;][vc_column_text css=\u00a0\u00bb.vc_custom_1752540791363{margin-bottom: 0px !important;}\u00a0\u00bb]<\/p>\n<h4 style=\"text-align: left;\"><a title=\"Cas Clinique 1\" href=\"https:\/\/www.atlasrleye.com\/fr\/manifestations-oculaires-de-la-ceroide-lipofuscinose-neuronale\/cas-clinique-1\/\">Cas Clinique 1<\/a><\/h4>\n<p>[\/vc_column_text][vc_gallery type=\u00a0\u00bbimage_grid\u00a0\u00bb images=\u00a0\u00bb21719,21718&Prime; img_size=\u00a0\u00bb150&Prime; css=\u00a0\u00bb.vc_custom_1752540829955{margin-bottom: 10px !important;margin-left: -20px !important;}\u00a0\u00bb][vc_row_inner][vc_column_inner width=\u00a0\u00bb1\/5&Prime; offset=\u00a0\u00bbvc_col-xs-1\/5&Prime;]<div class=\"aio-icon-component    style_1\"><div id=\"Info-box-wrap-9431\" class=\"aio-icon-box default-icon\" style=\"\"  ><div class=\"aio-icon-default\"><div class=\"ult-just-icon-wrapper  \"><div class=\"align-icon\" style=\"text-align:center;\">\n<div class=\"aio-icon none \"  style=\"color:#5f1b52;font-size:16px;display:inline-block;\">\n\t<i class=\"Defaults-calendar\"><\/i>\n<\/div><\/div><\/div><\/div><div class=\"aio-icon-header\" ><h3 class=\"aio-icon-title ult-responsive\"  data-ultimate-target='#Info-box-wrap-9431 .aio-icon-title'  data-responsive-json-new='{\"font-size\":\"\",\"line-height\":\"\"}'  style=\"color:#5f1b52;\">1<\/h3><\/div> <!-- header --><\/div> <!-- aio-icon-box --><\/div> <!-- aio-icon-component -->[\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/5&Prime; offset=\u00a0\u00bbvc_col-xs-1\/5&Prime;]<div class=\"aio-icon-component    style_1\"><div id=\"Info-box-wrap-2356\" class=\"aio-icon-box default-icon\" style=\"\"  ><div class=\"aio-icon-default\"><div class=\"ult-just-icon-wrapper  \"><div class=\"align-icon\" style=\"text-align:center;\">\n<div class=\"aio-icon none \"  style=\"color:#5f1b52;font-size:16px;display:inline-block;\">\n\t<i class=\"Defaults-camera\"><\/i>\n<\/div><\/div><\/div><\/div><div class=\"aio-icon-header\" ><h3 class=\"aio-icon-title ult-responsive\"  data-ultimate-target='#Info-box-wrap-2356 .aio-icon-title'  data-responsive-json-new='{\"font-size\":\"\",\"line-height\":\"\"}'  style=\"color:#5f1b52;\">6<\/h3><\/div> <!-- header --><\/div> <!-- aio-icon-box --><\/div> <!-- aio-icon-component -->[\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/5&Prime; offset=\u00a0\u00bbvc_col-xs-1\/5&Prime;]<div class=\"aio-icon-component    style_1\"><div id=\"Info-box-wrap-4003\" class=\"aio-icon-box default-icon\" style=\"\"  ><div class=\"aio-icon-default\"><div class=\"ult-just-icon-wrapper  \"><div class=\"align-icon\" style=\"text-align:center;\">\n<div class=\"aio-icon none \"  style=\"color:#5f1b52;font-size:16px;display:inline-block;\">\n\t<i class=\"Defaults-film\"><\/i>\n<\/div><\/div><\/div><\/div><div class=\"aio-icon-header\" ><h3 class=\"aio-icon-title ult-responsive\"  data-ultimate-target='#Info-box-wrap-4003 .aio-icon-title'  data-responsive-json-new='{\"font-size\":\"\",\"line-height\":\"\"}'  style=\"color:#5f1b52;\">0<\/h3><\/div> <!-- header --><\/div> <!-- aio-icon-box --><\/div> <!-- aio-icon-component -->[\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>[vc_row][vc_column][vc_custom_heading text=\u00a0\u00bbSYNDROMES ET DYSTROPHIES R\u00c9TINIENNES\u00a0\u00bb font_container=\u00a0\u00bbtag:h5|font_size:22px|text_align:center|color:%23000000&Prime; use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb link=\u00a0\u00bburl:https%3A%2F%2Fwww.atlasrleye.com%2Ffr%2Fatlas%2F%231636632807269-1444524b-3ed4|\u00a0\u00bb][vc_text_separator title=\u00a0\u00bb\u00a0\u00bb i_icon_fontawesome=\u00a0\u00bbfas fa-eye\u00a0\u00bb i_color=\u00a0\u00bbcustom\u00a0\u00bb i_size=\u00a0\u00bblg\u00a0\u00bb color=\u00a0\u00bbcustom\u00a0\u00bb border_width=\u00a0\u00bb8&Prime; css=\u00a0\u00bb.vc_custom_1637080776101{margin-bottom: -5px !important;}\u00a0\u00bb add_icon=\u00a0\u00bbtrue\u00a0\u00bb accent_color=\u00a0\u00bb#bababa\u00a0\u00bb i_custom_color=\u00a0\u00bb#bababa\u00a0\u00bb][vc_custom_heading source=\u00a0\u00bbpost_title\u00a0\u00bb font_container=\u00a0\u00bbtag:h1|font_size:44px|text_align:center|color:%235f1b52&Prime; use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb][\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb3\/4&Prime;][vc_column_text] La C\u00e9roide-Lipofuscinose Neuronale (NCL) est une maladie neurod\u00e9g\u00e9n\u00e9rative caus\u00e9e par une alt\u00e9ration du m\u00e9tabolisme des [&hellip;]<\/p>\n","protected":false},"author":3,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"footnotes":""},"class_list":["post-74243","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74243","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/users\/3"}],"replies":[{"embeddable":true,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/comments?post=74243"}],"version-history":[{"count":12,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74243\/revisions"}],"predecessor-version":[{"id":118096,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74243\/revisions\/118096"}],"wp:attachment":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/media?parent=74243"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}