{"id":475,"date":"2014-11-04T02:47:31","date_gmt":"2014-11-04T02:47:31","guid":{"rendered":"https:\/\/blogs.bmj.com\/jnnp\/?p=475"},"modified":"2014-11-04T02:47:31","modified_gmt":"2014-11-04T02:47:31","slug":"als-a-long-pre-clinical-period-does-it-start-at-conception","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/jnnp\/2014\/11\/04\/als-a-long-pre-clinical-period-does-it-start-at-conception\/","title":{"rendered":"ALS:  A long pre-clinical period.  Does it start at conception?"},"content":{"rendered":"<p>ALS is a rapidly progressive neurodegenerative disorder of the motor neurons with median survival of 3-5 years.\u00a0 The site of disease onset, and the timing of disease onset remain controversial, although<\/p>\n<p>a sudden and catastrophic degeneration of the motor neurons does occur.\u00a0 In this issue of JNNP a though provoking review by Eisen and colleagues suggests a long term pre clinicla period, perhaps stretching beck to the in utero period.\u00a0 In an<\/p>\n<p>accompanying letter,\u00a0 neuronal dysfunction (preceding degeneration) was reported in a zebra fish model.\u00a0 Interestingly, this dysfunction was slowed down by riluzole.\u00a0 The question remains as to whether we are too late by the time the patients present.\u00a0 But then, who should be treated\/monitored.\u00a0 The genetic cases, or all relatives of sporadic cases.\u00a0 Clearly there are more answers than questions, although headway has been made, and more research is needed.<\/p>\n<p>&nbsp;<\/p>\n<div class=\"cit-metadata\">\n<h4 class=\"cit-first-element cit-title-group\"><span class=\"cit-series-title\">Review<span class=\"cit-sep cit-sep-after-article-series-title\">:<\/span> <\/span>Amyotrophic lateral sclerosis: a long preclinical period?<\/h4>\n<ul class=\"cit-auth-list\">\n<li class=\"first-item\"><span class=\"cit-auth cit-auth-type-author\">Andrew Eisen<\/span><span class=\"cit-sep cit-sep-separator\">, <\/span><\/li>\n<li><span class=\"cit-auth cit-auth-type-author\">Matthew Kiernan<\/span><span class=\"cit-sep cit-sep-separator\">, <\/span><\/li>\n<li><span class=\"cit-auth cit-auth-type-author\">Hiroshi Mitsumoto<\/span><span class=\"cit-sep cit-sep-separator\">, <\/span><\/li>\n<li class=\"last-item\"><span class=\"cit-auth cit-auth-type-author\">Michael Swash<\/span><\/li>\n<\/ul>\n<p><cite><abbr class=\"site-title\" title=\"Journal of Neurology, Neurosurgery &amp; Psychiatry\">J Neurol Neurosurg Psychiatry<\/abbr> <span class=\"cit-print-date\">2014<span class=\"cit-sep cit-sep-after-article-print-date\">;<\/span><\/span><span class=\"cit-vol\">85<span class=\"cit-sep cit-sep-after-article-vol\">:<\/span><\/span><span class=\"cit-pages\"><span class=\"cit-first-page\">1232<\/span><span class=\"cit-sep\">&#8211;<\/span><span class=\"cit-last-page\">1238<\/span> <\/span><span class=\"cit-ahead-of-print-date\"><span class=\"cit-sep cit-sep-before-article-ahead-of-print-date\">Published Online First: <\/span>19 March 2014 <\/span><span class=\"cit-doi\"><span class=\"cit-sep cit-sep-before-article-doi\">doi:<\/span>10.1136\/jnnp-2013-307135 <\/span><\/cite><\/div>\n<div class=\"cit-metadata\"><\/div>\n<div class=\"cit-metadata\"><\/div>\n<div class=\"cit-metadata\">\n<div class=\"cit-metadata\">\n<h4 class=\"cit-first-element cit-title-group\"><span class=\"cit-series-title\">Letter<span class=\"cit-sep cit-sep-after-article-series-title\">:<\/span> <\/span>A zebrafish model exemplifies the long preclinical period of motor neuron disease<\/h4>\n<ul class=\"cit-auth-list\">\n<li class=\"first-item\"><span class=\"cit-auth cit-auth-type-author\">Tennore M Ramesh<\/span><span class=\"cit-sep cit-sep-separator\">, <\/span><\/li>\n<li><span class=\"cit-auth cit-auth-type-author\">Pamela J Shaw<\/span><span class=\"cit-sep cit-sep-separator\">, <\/span><\/li>\n<li class=\"last-item\"><span class=\"cit-auth cit-auth-type-author\">Jonathan McDearmid<\/span><\/li>\n<\/ul>\n<p><cite><abbr class=\"site-title\" title=\"Journal of Neurology, Neurosurgery &amp; Psychiatry\">J Neurol Neurosurg Psychiatry<\/abbr> <span class=\"cit-print-date\">2014<span class=\"cit-sep cit-sep-after-article-print-date\">;<\/span><\/span><span class=\"cit-vol\">85<span class=\"cit-sep cit-sep-after-article-vol\">:<\/span><\/span><span class=\"cit-pages\"><span class=\"cit-first-page\">1288<\/span><span class=\"cit-sep\">&#8211;<\/span><span class=\"cit-last-page\">1289<\/span> <\/span><span class=\"cit-ahead-of-print-date\"><span class=\"cit-sep cit-sep-before-article-ahead-of-print-date\">Published Online First: <\/span>26 June 2014 <\/span><span class=\"cit-doi\"><span class=\"cit-sep cit-sep-before-article-doi\">doi:<\/span>10.1136\/jnnp-2014-308288 <\/span><\/cite><\/div>\n<\/div>\n<p><!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>ALS is a rapidly progressive neurodegenerative disorder of the motor neurons with median survival of 3-5 years.\u00a0 The site of disease onset, and the timing of disease onset remain controversial, although a sudden and catastrophic degeneration of the motor neurons does occur.\u00a0 In this issue of JNNP a though provoking review by Eisen and colleagues [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/jnnp\/2014\/11\/04\/als-a-long-pre-clinical-period-does-it-start-at-conception\/\">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-475","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.5 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>ALS: A long pre-clinical period. Does it start at conception? - 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\/11\/04\/als-a-long-pre-clinical-period-does-it-start-at-conception\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"ALS: A long pre-clinical period. 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