{"id":405,"date":"2014-06-16T05:34:51","date_gmt":"2014-06-16T05:34:51","guid":{"rendered":"https:\/\/blogs.bmj.com\/jnnp\/?p=405"},"modified":"2014-06-16T05:35:02","modified_gmt":"2014-06-16T05:35:02","slug":"dystrophin-levels-in-beckers-md-a-cliff-effect","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/jnnp\/2014\/06\/16\/dystrophin-levels-in-beckers-md-a-cliff-effect\/","title":{"rendered":"Dystrophin levels in Beckers MD:  A cliff effect?"},"content":{"rendered":"<p>Becker muscular dystrophy (BMD) is an inherited muscle disorder caused by deficiency of dystrophin. The relationship between disease severity and dystrophin levels needs further assessment. In this issue of JNNP, a Dutch group reported an association between dystrophin level s and disease severity in BD. Specifically, dystrophin levels were not a major determinant of disease severity unless dystrophin levels &lt; 10% in muscles. Interestingly the study suggested that disease severity is more dependent on mutation site. So, where to from here, and what should be our treatment strategies?<\/p>\n<p>Read more http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract<\/p>\n<p>&nbsp;<\/p>\n<div id=\"slugline\"><cite><span id=\"article-slug-jnl-abbr\"><abbr class=\"slug-jnl-abbrev\" title=\"Journal of Neurology, Neurosurgery &amp; Psychiatry\"> Neurol Neurosurg Psychiatry<\/abbr> <\/span> <span class=\"slug-pub-date\">2014;<\/span><span class=\"slug-vol\">85<span class=\"cit-sep cit-sep-after-article-vol\">:<\/span><\/span><span class=\"slug-pages\">747-753 <\/span> <span class=\"slug-doi\" title=\"10.1136\/jnnp-2013-306350\"> doi:10.1136\/jnnp-2013-306350 <\/span> <\/cite><\/div>\n<ul class=\"subject-headings last-child\">\n<li>Neuromuscular<\/li>\n<\/ul>\n<ul class=\"series-titles\">\n<li>Research paper<\/li>\n<\/ul>\n<div class=\"article abstract-view \">\n<h1 id=\"article-title-1\">Dystrophin levels and clinical severity in Becker muscular dystrophy patients<\/h1>\n<div class=\"contributors\">\n<ol id=\"contrib-group-1\" class=\"contributor-list\">\n<li id=\"contrib-1\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=J+C+van+den+Bergen&amp;sortspec=date&amp;submit=Submit\">J C van den Bergen<\/a><\/span><a id=\"xref-aff-1-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-1\">1<\/a>,<\/li>\n<li id=\"contrib-2\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=B+H+Wokke&amp;sortspec=date&amp;submit=Submit\">B H Wokke<\/a><\/span><a id=\"xref-aff-1-2\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-1\">1<\/a>,<\/li>\n<li id=\"contrib-3\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=A+A+Janson&amp;sortspec=date&amp;submit=Submit\">A A Janson<\/a><\/span><a id=\"xref-aff-2-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-2\">2<\/a>,<\/li>\n<li id=\"contrib-4\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=S+G+van+Duinen&amp;sortspec=date&amp;submit=Submit\">S G van Duinen<\/a><\/span><a id=\"xref-aff-3-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-3\">3<\/a>,<\/li>\n<li id=\"contrib-5\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=M+A+Hulsker&amp;sortspec=date&amp;submit=Submit\">M A Hulsker<\/a><\/span><a id=\"xref-aff-4-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-4\">4<\/a>,<\/li>\n<li id=\"contrib-6\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=H+B+Ginjaar&amp;sortspec=date&amp;submit=Submit\">H B Ginjaar<\/a><\/span><a id=\"xref-aff-5-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-5\">5<\/a>,<\/li>\n<li id=\"contrib-7\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=J+C+van+Deutekom&amp;sortspec=date&amp;submit=Submit\">J C van Deutekom<\/a><\/span><a id=\"xref-aff-2-2\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-2\">2<\/a>,<\/li>\n<li id=\"contrib-8\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=A+Aartsma-Rus&amp;sortspec=date&amp;submit=Submit\">A Aartsma-Rus<\/a><\/span><a id=\"xref-aff-4-2\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-4\">4<\/a>,<\/li>\n<li id=\"contrib-9\" class=\"contributor\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=H+E+Kan&amp;sortspec=date&amp;submit=Submit\">H E Kan<\/a><\/span><a id=\"xref-aff-6-1\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-6\">6<\/a>,<\/li>\n<li id=\"contrib-10\" class=\"last\"><span class=\"name\"><a class=\"name-search\" href=\"http:\/\/jnnp.bmj.com\/search?author1=J+J+Verschuuren&amp;sortspec=date&amp;submit=Submit\">J J Verschuuren<\/a><\/span><a id=\"xref-aff-1-3\" class=\"xref-aff\" href=\"http:\/\/jnnp.bmj.com\/content\/85\/7\/747.abstract#aff-1\">1<\/a><\/li>\n<\/ol>\n<\/div>\n<\/div>\n<p><!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Becker muscular dystrophy (BMD) is an inherited muscle disorder caused by deficiency of dystrophin. The relationship between disease severity and dystrophin levels needs further assessment. In this issue of JNNP, a Dutch group reported an association between dystrophin level s and disease severity in BD. Specifically, dystrophin levels were not a major determinant of disease [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/jnnp\/2014\/06\/16\/dystrophin-levels-in-beckers-md-a-cliff-effect\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":160,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[],"class_list":["post-405","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.4 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Dystrophin levels in Beckers MD: A cliff effect? - JNNP blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/blogs.bmj.com\/jnnp\/2014\/06\/16\/dystrophin-levels-in-beckers-md-a-cliff-effect\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Dystrophin levels in Beckers MD: A cliff effect? - JNNP blog\" \/>\n<meta property=\"og:description\" content=\"Becker muscular dystrophy (BMD) is an inherited muscle disorder caused by deficiency of dystrophin. 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