{"id":1922,"date":"2011-10-01T01:00:00","date_gmt":"2011-10-01T00:00:00","guid":{"rendered":"https:\/\/angh.net\/abstracts\/transplantation-hepatique-pour-degenerescence-hepato-cerebrale-quand-le-foie-guerit-la-tete\/"},"modified":"2018-10-20T18:39:58","modified_gmt":"2018-10-20T16:39:58","slug":"transplantation-hepatique-pour-degenerescence-hepato-cerebrale-quand-le-foie-guerit-la-tete","status":"publish","type":"post","link":"https:\/\/angh.net\/abstracts\/transplantation-hepatique-pour-degenerescence-hepato-cerebrale-quand-le-foie-guerit-la-tete\/","title":{"rendered":"Transplantation h\u00e9patique pour d\u00e9g\u00e9n\u00e9rescence h\u00e9pato-c\u00e9r\u00e9brale : Quand le foie gu\u00e9rit la t\u00eate."},"content":{"rendered":"<p><strong>2011<\/strong><\/p>\n<p><em>Alix Portal, Isabelle Rosa, Christian Meyrignac, Camille Barrault, Laurent Costes, Thierry Lons, Herv\u00e9 Hag\u00e8ge<\/em><\/p>\n<p><strong>H\u00e9patologie <\/strong>&#8211; \u00a02011-05-12 &#8211;\u00a0CO &#8211;<\/p>\n<p>________________________________<\/p>\n<p>Monsieur M, 56 ans, a comme ant\u00e9c\u00e9dent un diab\u00e8te de type 2 compliqu\u00e9 d\u2019une r\u00e9tinopathie diab\u00e9tique. En 2006, est d\u00e9couverte une cirrhose devant une d\u00e9compensation oed\u00e9mato-ascitique. Le bilan initial est en faveur d\u2019une origine m\u00e9tabolique (pas d\u2019alcool, bilan viral et auto-immun n\u00e9gatif, c\u00e9ruloplasmine et cupr\u00e9mie normales, PBH en faveur), Le score de  Child Pugh \u00e9tait B6, avec une hypertension portale ( VO de grade 3). En octobre 2008, il existe une d\u00e9gradation progressive de la fonction h\u00e9patique avec ict\u00e8re, baisse du TP \u00e0 50% et un \u00e9pisode d\u2019enc\u00e9phalopathie h\u00e9patique sans \u00e9tiologie retrouv\u00e9e. Secondairement,  apparaissait une symptomatologie neurologique d\u2019aggravation rapide constitu\u00e9e d\u2019un syndrome extra-pyramidal (dystonie, dysarthrie, trouble de la marche), de mouvements chor\u00e9iques des quatre membres et de la face associ\u00e9 \u00e0 des troubles comportementaux (syndrome frontal). La recherche exhaustive d\u2019une maladie de Wilson s\u2019est av\u00e9r\u00e9e n\u00e9gative \u00e0 plusieurs reprises. L\u2019IRM c\u00e9r\u00e9brale retrouvait un hypersignal T1 des pallidum associ\u00e9 \u00e0 des l\u00e9sions sous-tentorielles sym\u00e9triques en hypersignal FLAIR ; l\u2019EEG montrait des stigmates d\u2019enc\u00e9phalopathie h\u00e9patique s\u00e9v\u00e8re. Apr\u00e8s discussion avec les neurologues, le diagnostic retenu \u00e9tait une d\u00e9g\u00e9n\u00e9rescence h\u00e9pato-c\u00e9r\u00e9brale.<br \/>\nLa progression rapide de la maladie neurologique (chutes \u00e0 r\u00e9p\u00e9tition compliqu\u00e9es d\u2019un h\u00e9matome sous-dural, perte d\u2019autonomie avec d\u00e9placement en fauteuil roulant), faisait poser l\u2019indication d\u2019une transplantation h\u00e9patique orthotopique dont le patient b\u00e9n\u00e9ficiait le 18 mars 2010. L\u2019am\u00e9lioration de la symptomatologie neurologique e\u00e9tait spectaculaire avec un retour \u00e0 l\u2019autonomie compl\u00e8te d\u00e8s le troisi\u00e8me mois post-greffe (marche quasi normalis\u00e9e, disparition de la dysarthrie et des mouvements chor\u00e9iques, syndrome c\u00e9r\u00e9belleux a minima avec \u00e9largissement du polygone de sustentation). Actuellement (14 mois post greffe) aucun sympt\u00f4me neurologique n\u2019est r\u00e9apparu.<br \/>\nLe syndrome de d\u00e9g\u00e9n\u00e9rescence h\u00e9pato-c\u00e9r\u00e9brale est un syndrome rare dont il n\u2019existe que quelques cas d\u00e9crits dans la litt\u00e9rature. La transplantation h\u00e9patique orthotopique peut \u00eatre propos\u00e9e comme traitement de ce syndrome.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>2011 Alix Portal, Isabelle Rosa, Christian Meyrignac, Camille Barrault, Laurent Costes, Thierry Lons, Herv\u00e9 Hag\u00e8ge H\u00e9patologie &#8211; \u00a02011-05-12 &#8211;\u00a0CO &#8211; ________________________________ Monsieur M, 56 ans, a comme ant\u00e9c\u00e9dent un diab\u00e8te de type 2 compliqu\u00e9 d\u2019une r\u00e9tinopathie diab\u00e9tique. En 2006, est d\u00e9couverte une cirrhose devant une d\u00e9compensation oed\u00e9mato-ascitique. Le bilan initial est en faveur d\u2019une origine [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[3],"tags":[22],"class_list":["post-1922","post","type-post","status-publish","format-standard","hentry","category-hepatologie","tag-22"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.2 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Transplantation h\u00e9patique pour d\u00e9g\u00e9n\u00e9rescence h\u00e9pato-c\u00e9r\u00e9brale : Quand le foie gu\u00e9rit la t\u00eate. - Abstracts des congr\u00e8s de l&#039;ANGH<\/title>\n<meta name=\"description\" content=\"ANGH R\u00e9sum\u00e9s congr\u00e8s H\u00e9patologie Gastroent\u00e9romogie Hepatology Gastroenterology Etudes cliniques Clinical study\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/angh.net\/abstracts\/transplantation-hepatique-pour-degenerescence-hepato-cerebrale-quand-le-foie-guerit-la-tete\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Transplantation h\u00e9patique pour d\u00e9g\u00e9n\u00e9rescence h\u00e9pato-c\u00e9r\u00e9brale : Quand le foie gu\u00e9rit la t\u00eate. - 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