{"id":74453,"date":"2021-11-23T16:11:58","date_gmt":"2021-11-23T16:11:58","guid":{"rendered":"https:\/\/www.atlasrleye.com\/maladie-de-best\/"},"modified":"2025-07-15T10:39:12","modified_gmt":"2025-07-15T10:39:12","slug":"maladie-de-best","status":"publish","type":"page","link":"https:\/\/www.atlasrleye.com\/fr\/maladie-de-best\/","title":{"rendered":"MALADIE DE BEST"},"content":{"rendered":"

[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″ 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″ 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″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb]

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La maladie de Best (dystrophie maculaire vitelliforme) est une maculopathie de transmission autosomique dominant caus\u00e9e par mutations dans le g\u00e8ne codant pour la prot\u00e9ine Bestrophine.<\/p>\n

Cette mutation provoque une accumulation sous-r\u00e9tinienne maculaire de lipofuscine (pigment jaune), donnant un aspect de jaune d\u2019\u0153uf \u00e0 la macula. Bien que les signes du fond d’\u0153il soient g\u00e9n\u00e9ralement confin\u00e9s \u00e0 la r\u00e9gion maculaire, la maladie pr\u00e9sente une expressivit\u00e9 tr\u00e8s variable et le fond est parfois normal. L\u2019\u00e9lectro-oculographie est toujours anormale dans la maladie de Best et sert comme marqueur pour elle, m\u00eame chez les patients asymptomatiques avec un fond d’\u0153il normale et qui montrent une perte importante de r\u00e9ponse \u00e0 la lumi\u00e8re.<\/p>\n

La maladie progresse lentement et finit par entra\u00eener une atrophie de l\u2019\u00e9pith\u00e9lium pigment\u00e9 de la r\u00e9tine et des photor\u00e9cepteurs, alt\u00e9rant gravement la vision centrale. L\u2019autofluorescence de fond est utile pour d\u00e9tecter l\u2019accumulation de lipofuscine qui est hyperautofluorescente. Les patients se plaignent d\u2019une vision floue, d\u2019une perte d\u2019acuit\u00e9 visuelle ou d\u2019une m\u00e9tamorphopsie, et les sympt\u00f4mes s\u2019aggravent si la maladie progresse vers l\u2019\u00e9tat atrophique.<\/p>\n

Des mutations du g\u00e8ne VMD2 codant pour la bestrofine, cartographi\u00e9 \u00e0 11q12-q13.1, ont \u00e9t\u00e9 associ\u00e9es \u00e0 la maladie de Best. \u00c0 ce jour, il n\u2019existe aucun traitement pour cette maladie.<\/p>\n

Diagnostic diff\u00e9rentiel: dystrophie vitelliforme maculaire adulte (dystrophie standard), drusen laminaire basal, maculopathie vitelliforme exsudative aigu\u00eb, maladie de Stargardt avec des taches larges centrales et t\u00e9langiectasies maculaires idiopathiques.<\/p>\n<\/div>\n

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Dystrophie maculaire vitelliforme de l’adulte (MMA)<\/p>\n

La dystrophie maculaire vitelliforme de l’adulte pr\u00e9sente des l\u00e9sions plus petites que la maladie de Best, sans interruption ni stratification du pigment jaun\u00e2tre, chez les patients \u00e2g\u00e9s. Il se caract\u00e9rise par des l\u00e9sions entour\u00e9es de bords sombres et une agglom\u00e9ration pigmentaire bilat\u00e9rale et sym\u00e9trique. Elle commence entre 30 et 50 ans avec une vision et des m\u00e9tamorphopsies peu affect\u00e9es, souvent avec des sympt\u00f4mes unilat\u00e9raux. Vous avez un bon pronostic.<\/p>\n<\/div>\n

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Cas Cliniques<\/span><\/strong><\/h1>\n

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Cas Clinique 1<\/a><\/h4>\n

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0<\/h3><\/div> <\/div> <\/div> [\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"

[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″ 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″ 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″ use_theme_fonts=\u00a0\u00bbyes\u00a0\u00bb][\/vc_column][\/vc_row][vc_row][vc_column width=\u00a0\u00bb3\/4″][vc_column_text] La maladie de Best (dystrophie maculaire vitelliforme) est une maculopathie de transmission autosomique dominant caus\u00e9e […]<\/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-74453","page","type-page","status-publish","hentry"],"_links":{"self":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74453","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=74453"}],"version-history":[{"count":31,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74453\/revisions"}],"predecessor-version":[{"id":118114,"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/pages\/74453\/revisions\/118114"}],"wp:attachment":[{"href":"https:\/\/www.atlasrleye.com\/fr\/wp-json\/wp\/v2\/media?parent=74453"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}