{"id":345,"date":"2014-03-27T15:20:27","date_gmt":"2014-03-27T14:20:27","guid":{"rendered":"http:\/\/s519078666.onlinehome.fr\/?page_id=345"},"modified":"2014-11-17T16:12:08","modified_gmt":"2014-11-17T15:12:08","slug":"les-formes-cliniques-et-leur-evolution","status":"publish","type":"page","link":"http:\/\/www.stargardt.fr\/test\/comprendre-la-maladie-de-stargardt-maladie-orpheline\/les-formes-cliniques-et-leur-evolution\/","title":{"rendered":"Les formes cliniques et leur \u00e9volution"},"content":{"rendered":"<h2>\u00c2ge et alt\u00e9ration de la vision<\/h2>\n<p><strong>Dans la maladie de Stargardt, l\u2019atteinte cellulaire porte sur les photor\u00e9cepteurs de la macula constitu\u00e9e principalement de photor\u00e9cepteurs de type c\u00f4nes.<\/strong><\/p>\n<p>Il en r\u00e9sulte une perte de la vision centrale (finesse et d\u00e9tails) des \u00e9l\u00e9ments situ\u00e9s dans l\u2019axe du regard ainsi que des troubles de la vision des couleurs. Les personnes expriment aussi des difficult\u00e9s \u00e0 voir en lumi\u00e8re tamis\u00e9e ou en lumi\u00e8re nocturne.<\/p>\n<p>Il existe diff\u00e9rentes formes de la maladie de Stargardt, de m\u00eame origine g\u00e9n\u00e9tique dont seules les mutations du g\u00e8ne ABCA4 sont diff\u00e9rentes. Les principales sont\u00a0:<\/p>\n<h3>Forme typique de l\u2019enfant\u00a0:<\/h3>\n<p>forme la plus fr\u00e9quente. Les signes apparaissent dans l\u2019enfance (6-15 ans) le plus souvent \u00e0 l\u2019adolescence\u00a0: difficult\u00e9s \u00e0 la lecture, \u00e0 l\u2019\u00e9criture, difficult\u00e9 \u00e0 regarder la TV, mauvaise perception des visages, d\u00e9fauts de perception de la couleur rouge ou verte, crainte de la lumi\u00e8re li\u00e9e \u00e0 la g\u00eane qu\u2019elle provoque\u2026 L\u2019acuit\u00e9 visuelle des deux yeux (bilat\u00e9rale) baisse rapidement et progressivement et passe de 1\/10\u00e8me \u00e0 1\/20\u00e8me en 2 ou 3 ans.<\/p>\n<h3>Forme tardive de l\u2019adulte\u00a0:<\/h3>\n<p>appel\u00e9e fundus flavimaculatus. Elle se manifeste plus tardivement (20-60 ans) et dont l\u2019\u00e9volution est plus lente et moins s\u00e9v\u00e8re. Au d\u00e9but de la maladie, la r\u00e9gion centrale de la r\u00e9tine n\u2019est pas alt\u00e9r\u00e9e.<\/p>\n<p>Des formes s\u00e9v\u00e8res mais rares avec atteinte des r\u00e9gions p\u00e9riph\u00e9riques de la r\u00e9tine (champ visuel alt\u00e9r\u00e9) ont \u00e9t\u00e9 d\u00e9crites.<\/p>\n<h4>R\u00e9tinopathies pigmentaires<\/h4>\n<p><a href=\"http:\/\/s519078666.onlinehome.fr\/wp-content\/uploads\/2014\/03\/Alteration-Batonnets-Cones.jpg\" rel=\"PrettyPhoto[345]\"><img loading=\"lazy\" class=\"alignnone  wp-image-487\" title=\"Alt\u00e9ration B\u00e2tonnets C\u00f4nes\" src=\"http:\/\/s519078666.onlinehome.fr\/wp-content\/uploads\/2014\/03\/Alteration-Batonnets-Cones-300x240.jpg\" alt=\"Alt\u00e9ration B\u00e2tonnets C\u00f4nes\" width=\"250\" height=\"200\" srcset=\"http:\/\/www.stargardt.fr\/test\/wp-content\/uploads\/sites\/5\/2014\/03\/Alteration-Batonnets-Cones-300x240.jpg 300w, http:\/\/www.stargardt.fr\/test\/wp-content\/uploads\/sites\/5\/2014\/03\/Alteration-Batonnets-Cones.jpg 317w\" sizes=\"(max-width: 250px) 100vw, 250px\" \/><\/a><a href=\"http:\/\/s519078666.onlinehome.fr\/wp-content\/uploads\/2014\/03\/Alteration-Cones-Batonnets.jpg\" rel=\"PrettyPhoto[345]\">\u00a0 <\/a><a href=\"http:\/\/s519078666.onlinehome.fr\/wp-content\/uploads\/2014\/03\/Alteration-Cones-Batonnets.jpg\" rel=\"PrettyPhoto[345]\"><img loading=\"lazy\" class=\"alignnone wp-image-486 size-medium\" title=\"Alt\u00e9ration C\u00f4nes B\u00e2tonnets \" src=\"http:\/\/s519078666.onlinehome.fr\/wp-content\/uploads\/2014\/03\/Alteration-Cones-Batonnets-300x200.jpg\" alt=\"Perte de la vision centrale : Alt\u00e9ration C\u00f4nes B\u00e2tonnets \" width=\"300\" height=\"200\" srcset=\"http:\/\/www.stargardt.fr\/test\/wp-content\/uploads\/sites\/5\/2014\/03\/Alteration-Cones-Batonnets-300x200.jpg 300w, http:\/\/www.stargardt.fr\/test\/wp-content\/uploads\/sites\/5\/2014\/03\/Alteration-Cones-Batonnets.jpg 381w\" sizes=\"(max-width: 300px) 100vw, 300px\" \/><\/a><\/p>\n<p style=\"text-align: left;\">Alt\u00e9ration B\u00e2tonnets-C\u00f4nes \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0 \u00a0<strong>Alt\u00e9ration C\u00f4nes-B\u00e2tonnets\u00a0<\/strong><strong>= Maladie de Stargardt<\/strong><\/p>\n<div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img src=\"http:\/\/static.hupso.com\/share\/buttons\/lang\/fr\/share.png\" rel=\"PrettyPhoto[345]\" style=\"border:0px; padding-top: 5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Google Plus\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"big\";var hupso_image_folder_url = \"\";var hupso_url_t=\"\";var hupso_title_t=\"Les%20formes%20cliniques%20et%20leur%20%C3%A9volution\";<\/script><script type=\"text\/javascript\" src=\"http:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>","protected":false},"excerpt":{"rendered":"<p>\u00c2ge et alt\u00e9ration de la vision Dans la maladie de Stargardt, l\u2019atteinte cellulaire porte sur les photor\u00e9cepteurs de la macula constitu\u00e9e principalement de photor\u00e9cepteurs de type c\u00f4nes. Il en r\u00e9sulte une perte de la vision centrale (finesse et d\u00e9tails) des \u00e9l\u00e9ments situ\u00e9s dans l\u2019axe du regard ainsi que des troubles de la vision des couleurs&#8230;.<\/p>\n<div style=\"padding-bottom:20px; padding-top:10px;\" class=\"hupso-share-buttons\"><!-- Hupso Share Buttons - https:\/\/www.hupso.com\/share\/ --><a class=\"hupso_toolbar\" href=\"https:\/\/www.hupso.com\/share\/\"><img src=\"http:\/\/static.hupso.com\/share\/buttons\/lang\/fr\/share.png\" style=\"border:0px; padding-top: 5px; float:left;\" alt=\"Share Button\"\/><\/a><script type=\"text\/javascript\">var hupso_services_t=new Array(\"Twitter\",\"Facebook\",\"Google Plus\");var hupso_background_t=\"#EAF4FF\";var hupso_border_t=\"#66CCFF\";var hupso_toolbar_size_t=\"big\";var hupso_image_folder_url = \"\";var hupso_url_t=\"\";var hupso_title_t=\"Les%20formes%20cliniques%20et%20leur%20%C3%A9volution\";<\/script><script type=\"text\/javascript\" src=\"http:\/\/static.hupso.com\/share\/js\/share_toolbar.js\"><\/script><!-- Hupso Share Buttons --><\/div>","protected":false},"author":2,"featured_media":0,"parent":301,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v19.4 - 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