{"id":4444,"date":"2010-09-06T18:42:50","date_gmt":"2010-09-06T22:42:50","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=4444"},"modified":"2013-06-12T22:22:32","modified_gmt":"2013-06-13T02:22:32","slug":"amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/","title":{"rendered":"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s Disease)"},"content":{"rendered":"

Pathophysiology<\/strong><\/p>\n

a group of disorders with progressive upper and lower motor neuron disease causing progressive motor weakness<\/p>\n

Signs and Symptoms<\/strong><\/p>\n

1)<\/strong> lower motor neuron signs are symmetric muscle atrophy and fasciculation (rapid and irregular contractions that do not move the limb) 2)<\/strong> upper motor neuron signs are spasticity and hyperreflexia 3)<\/strong> first sign is usually wasting and weakness of hand muscles
\n
\nHistology\/Gross Pathology<\/strong><\/p>\n

1)<\/strong> hallmark is loss of large motor neurons 2)<\/strong> progressive degeneration of anterior horn motor cells accompanied by gliosis 3)<\/strong> loss of myelinated fibers laterally in corticospinal tracts 4)<\/strong> degeneration of motor nuclei in brainstem (especially hypoglossal nuclei, ambiguous and motor trigeminal nuclei 5)<\/strong> degeneration of upper motor neurons of cerebral cortex 6) atrophy of skeletal muscle fibers<\/p>\n

Associated Conditions<\/strong><\/p>\n

death occurs from respiratory failure and recurrent bouts of pneumonia (often owing to aspiration)
\n
\nInheritance\/Epidemiology<\/strong><\/p>\n

1)<\/strong> most common form of motor neuron disease 2)<\/strong> familial – 5% of cases with autosomal dominant inheritance; affected gene (chromosome 21) controls copper-zinc superoxide dismutase 3)<\/strong> duration from onset to death is usually several years 4)<\/strong> worldwide incidence 1\/100,000 5)<\/strong> peaks at ages 40-50 years 6)<\/strong> male to female; 2:1<\/p>\n

Treatment<\/strong><\/p>\n

1)<\/strong> scattered early anecdotal reports of antibiotic treatment of patients who have improved or stabilized on ceftriaxone and penicillin have led to more in depth studies 2)<\/strong> some experienced ALS neurologists think that ceftriaxone may interact in a beneficial way with select neurotransmitters 3)<\/strong> a minority of physicians think some subsets of ALS may in fact be misdiagnosed Borrelia infection (Lyme disease)<\/p>\n

Tips for USMLE<\/strong><\/p>\n

1)<\/strong> lower motor neuron signs are due to loss of anterior horn cells; upper motor neuron signs are due to loss of corticospinal tracts 2)<\/strong> look for a 45-year old auto mechanic who presents with a complaint of constantly dropping his tools from his right hand.<\/p>\n

Relate<\/strong>d Posts<\/strong><\/p>\n

Former NFL Fullback Diagnosed With Lou Gehrig’s Disease<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology a group of disorders with progressive upper and lower motor neuron disease causing progressive motor weakness Signs and Symptoms 1) lower motor neuron signs are symmetric muscle atrophy and fasciculation (rapid and irregular contractions that do not move the limb) 2) upper motor neuron signs are spasticity and hyperreflexia 3) first sign is usually […]<\/p>\n","protected":false},"author":1,"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,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[5103,63],"tags":[2849,2788,3111,3112,3110],"yoast_head":"\nAmyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information 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:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information Blog\" \/>\n<meta property=\"og:description\" content=\"Pathophysiology a group of disorders with progressive upper and lower motor neuron disease causing progressive motor weakness Signs and Symptoms 1) lower motor neuron signs are symmetric muscle atrophy and fasciculation (rapid and irregular contractions that do not move the limb) 2) upper motor neuron signs are spasticity and hyperreflexia 3) first sign is usually […]\" \/>\n<meta property=\"og:url\" content=\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/\" \/>\n<meta property=\"og:site_name\" content=\"InsideSurgery Medical Information Blog\" \/>\n<meta property=\"article:published_time\" content=\"2010-09-06T22:42:50+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2013-06-13T02:22:32+00:00\" \/>\n<meta name=\"author\" content=\"Editor\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"Editor\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\/\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/\",\"url\":\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/\",\"name\":\"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information Blog\",\"isPartOf\":{\"@id\":\"https:\/\/insidesurgery.com\/#website\"},\"datePublished\":\"2010-09-06T22:42:50+00:00\",\"dateModified\":\"2013-06-13T02:22:32+00:00\",\"author\":{\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\"},\"breadcrumb\":{\"@id\":\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/#breadcrumb\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"ReadAction\",\"target\":[\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/\"]}]},{\"@type\":\"BreadcrumbList\",\"@id\":\"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/#breadcrumb\",\"itemListElement\":[{\"@type\":\"ListItem\",\"position\":1,\"name\":\"Home\",\"item\":\"https:\/\/insidesurgery.com\/\"},{\"@type\":\"ListItem\",\"position\":2,\"name\":\"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s Disease)\"}]},{\"@type\":\"WebSite\",\"@id\":\"https:\/\/insidesurgery.com\/#website\",\"url\":\"https:\/\/insidesurgery.com\/\",\"name\":\"InsideSurgery Medical Information Blog\",\"description\":\"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news\",\"potentialAction\":[{\"@type\":\"SearchAction\",\"target\":{\"@type\":\"EntryPoint\",\"urlTemplate\":\"https:\/\/insidesurgery.com\/?s={search_term_string}\"},\"query-input\":\"required name=search_term_string\"}],\"inLanguage\":\"en-US\"},{\"@type\":\"Person\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594\",\"name\":\"Editor\",\"image\":{\"@type\":\"ImageObject\",\"inLanguage\":\"en-US\",\"@id\":\"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/\",\"url\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"contentUrl\":\"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g\",\"caption\":\"Editor\"}}]}<\/script>\n<!-- \/ Yoast SEO plugin. -->","yoast_head_json":{"title":"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information Blog","robots":{"index":"index","follow":"follow","max-snippet":"max-snippet:-1","max-image-preview":"max-image-preview:large","max-video-preview":"max-video-preview:-1"},"canonical":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/","og_locale":"en_US","og_type":"article","og_title":"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information Blog","og_description":"Pathophysiology a group of disorders with progressive upper and lower motor neuron disease causing progressive motor weakness Signs and Symptoms 1) lower motor neuron signs are symmetric muscle atrophy and fasciculation (rapid and irregular contractions that do not move the limb) 2) upper motor neuron signs are spasticity and hyperreflexia 3) first sign is usually […]","og_url":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/","og_site_name":"InsideSurgery Medical Information Blog","article_published_time":"2010-09-06T22:42:50+00:00","article_modified_time":"2013-06-13T02:22:32+00:00","author":"Editor","twitter_misc":{"Written by":"Editor","Est. reading time":"1 minute"},"schema":{"@context":"https:\/\/schema.org","@graph":[{"@type":"WebPage","@id":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/","url":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/","name":"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig's Disease) - InsideSurgery Medical Information Blog","isPartOf":{"@id":"https:\/\/insidesurgery.com\/#website"},"datePublished":"2010-09-06T22:42:50+00:00","dateModified":"2013-06-13T02:22:32+00:00","author":{"@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594"},"breadcrumb":{"@id":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/#breadcrumb"},"inLanguage":"en-US","potentialAction":[{"@type":"ReadAction","target":["https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/"]}]},{"@type":"BreadcrumbList","@id":"https:\/\/insidesurgery.com\/2010\/09\/amyotrophic-lateral-sclerosis-als-lou-gehrigs-disease\/#breadcrumb","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https:\/\/insidesurgery.com\/"},{"@type":"ListItem","position":2,"name":"Amyotrophic Lateral Sclerosis (ALS, Lou Gehrig’s Disease)"}]},{"@type":"WebSite","@id":"https:\/\/insidesurgery.com\/#website","url":"https:\/\/insidesurgery.com\/","name":"InsideSurgery Medical Information Blog","description":"Details of surgical procedures, pathophysiology, signs and symptoms, and treatment of medical diseases,medical and surgical eponyms, and surgeons and surgery in the news","potentialAction":[{"@type":"SearchAction","target":{"@type":"EntryPoint","urlTemplate":"https:\/\/insidesurgery.com\/?s={search_term_string}"},"query-input":"required name=search_term_string"}],"inLanguage":"en-US"},{"@type":"Person","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/80c1e5c67f8a2c348e38e202270e8594","name":"Editor","image":{"@type":"ImageObject","inLanguage":"en-US","@id":"https:\/\/insidesurgery.com\/#\/schema\/person\/image\/","url":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","contentUrl":"https:\/\/secure.gravatar.com\/avatar\/41c3c845307fcd3eff88632d6ccaf656?s=96&d=mm&r=g","caption":"Editor"}}]}},"_links":{"self":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/4444"}],"collection":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/comments?post=4444"}],"version-history":[{"count":0,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/posts\/4444\/revisions"}],"wp:attachment":[{"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/media?parent=4444"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/categories?post=4444"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/insidesurgery.com\/wp-json\/wp\/v2\/tags?post=4444"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}