{"id":4175,"date":"2025-03-18T08:53:52","date_gmt":"2025-03-18T07:53:52","guid":{"rendered":"https:\/\/kineaphp.fr\/?p=4175"},"modified":"2026-01-03T11:53:42","modified_gmt":"2026-01-03T10:53:42","slug":"sclerose-laterale-amyotrophique-sla","status":"publish","type":"post","link":"https:\/\/kineaphp.fr\/?p=4175","title":{"rendered":"Scl\u00e9rose lat\u00e9rale amyotrophique (SLA)"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">G\u00e9n\u00e9ralit\u00e9s<\/h2>\n\n\n\n<p>SLA : maladie de la commande motrice. Perte s\u00e9lective en motoneurones au niveau :<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Du cortex moteur (MTN centraux)<\/li>\n\n\n\n<li>Des noyaux du bulbes (faisceau cortico-nucl\u00e9aire : synapse au niveau des noyaux moteurs des nerfs cr\u00e2niens)<\/li>\n\n\n\n<li>De la corne ant\u00e9rieure de la moelle \u00e9pini\u00e8re (MTN p\u00e9riph\u00e9rique)<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">S\u00e9miologie<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">D\u00e9ficit moteur<\/h3>\n\n\n\n<p>Atteinte des MNC et MNP dans un ou plusieurs des 4 territoires anatomique :<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Bulbaire : parole, d\u00e9glutition<\/li>\n\n\n\n<li>Cervical : MS, respiratoire<\/li>\n\n\n\n<li>Lombaire : MI<\/li>\n\n\n\n<li>Thoracique\/respiration : camptocormie<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Signe cardinal de la SLA<\/h3>\n\n\n\n<p>Association des signes cliniques d&rsquo;atteinte <\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>des MTN centraux : syndrome pyramidal, affect pseudo-bulbaire (labilit\u00e9 \u00e9motionnelle : tr\u00e8s \u00e9motif)<\/li>\n\n\n\n<li>des MTN p\u00e9riph\u00e9riques : amyotrophie, fasciculations, diminution ou abolition ROT<\/li>\n<\/ul>\n\n\n\n<p>D\u00e9but de la maladie<\/p>\n\n\n\n<p>Site de d\u00e9but : 1\/3 bulbaire, 2\/3 spinal<br>Signe cardinal : d\u00e9ficit moteur avec localisation variable<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Membres sup\u00e9rieurs : distal (main++) ou proximal<\/li>\n\n\n\n<li>Membres inf\u00e9rieurs : steppage++<\/li>\n\n\n\n<li>Muscles du cou : t\u00eate tombante, lourde<\/li>\n\n\n\n<li>Sph\u00e8re bulbaire : dysarthrie, trouble de la d\u00e9glutition<\/li>\n\n\n\n<li>Respiratoire (dysfonction diaphragmatique : dyspn\u00e9e d&rsquo;effort, orthopn\u00e9e<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Sympt\u00f4mes non moteurs absents<\/h3>\n\n\n\n<p>Absence de :<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Signes sensitifs<\/li>\n\n\n\n<li>Signes c\u00e9rebelleux<\/li>\n\n\n\n<li>Atteinte sphinct\u00e9rienne<\/li>\n\n\n\n<li>Atteinte occulomotrice<\/li>\n\n\n\n<li>Troubles cognitifs (rares)<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">Diagnostic<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Diagnostic syndromique<\/h3>\n\n\n\n<p><strong>Pas de marqueur sp\u00e9cifique de la maladie<\/strong><br>Bilan diagnostic minimal : <\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>ENMG <\/strong>: confirme atteinte MNP<\/li>\n\n\n\n<li><strong>IRM c\u00e9r\u00e9brale ou m\u00e9dullaire<\/strong> : permet d&rsquo;exclure diagnostics diff\u00e9rentiels<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Exclusions des diagnostics diff\u00e9rentiels : ALS mimics<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li><strong>Tableau d&rsquo;atteinte isol\u00e9e du MNC <\/strong>: forme progressive de SEP, parasie spastique h\u00e9r\u00e9ditaire<\/li>\n\n\n\n<li><strong>Atteinte m\u00e9dullaire focale <\/strong>: my\u00e9lopathie cervicarthrosique (compression m\u00e9dullaire cervicale), synringomy\u00e9lie<\/li>\n\n\n\n<li><strong>Tableau d&rsquo;atteinte isol\u00e9e du MNP<\/strong> : myosite \u00e0 inclusion, neuropathie multifocale<\/li>\n\n\n\n<li><strong>Symptomatologie bulbaire pure<\/strong> : myasth\u00e9nie bulbaire, tumeur du TC<\/li>\n\n\n\n<li><strong>Atteinte du MN secondaires\/autres<\/strong> : infection VIH, lymphome<\/li>\n<\/ul>\n\n\n\n<hr class=\"wp-block-separator has-alpha-channel-opacity\"\/>\n\n\n\n<h2 class=\"wp-block-heading\">Pronostic<\/h2>\n\n\n\n<h3 class=\"wp-block-heading\">Evolution<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Absence de r\u00e9cup\u00e9ration de la fonction motrice perdue<\/li>\n\n\n\n<li>Aggravation pr\u00e9visible de la perte de force et diffusion \u00e0 d&rsquo;autres territoires<\/li>\n\n\n\n<li>Vitesse et topographie de l&rsquo;aggravation sont variables et non pr\u00e9dictibles<\/li>\n\n\n\n<li>M\u00e9diane de survie : 20 \u00e0 48 mois, 10\/20% de survie \u00e0 5 ans, 5\/10% de survie \u00e0 10 ans<\/li>\n<\/ul>\n\n\n\n<h3 class=\"wp-block-heading\">Facteurs de mauvais pronostic<\/h3>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Age : plus l&rsquo;\u00e2ge est \u00e9lev\u00e9 au moment du diagnostic, plus la dur\u00e9e de survie sera courte<\/li>\n\n\n\n<li>Baisse de la capacit\u00e9 vitale inf\u00e9rieure \u00e0 70% (atteinte respiratoire)<\/li>\n\n\n\n<li>Perte de poids et baisse de l&rsquo;IMC : un des plus mauvais facteurs pronostics<\/li>\n\n\n\n<li>Atteinte bulbaire ou respiratoire initiale<\/li>\n\n\n\n<li>Pente rapide de d\u00e9gradation de la fonction musculaire<\/li>\n<\/ul>\n","protected":false},"excerpt":{"rendered":"<p>G\u00e9n\u00e9ralit\u00e9s SLA : maladie de la commande motrice. Perte s\u00e9lective en motoneurones au niveau : S\u00e9miologie D\u00e9ficit moteur Atteinte des MNC et MNP dans un ou plusieurs des 4 territoires anatomique : Signe cardinal de&hellip;<\/p>\n<p class=\"more-link-wrapper\"><a href=\"https:\/\/kineaphp.fr\/?p=4175\" class=\"more-link\">Continue Reading<span class=\"screen-reader-text\"> \u00ab\u00a0Scl\u00e9rose lat\u00e9rale amyotrophique (SLA)\u00a0\u00bb<\/span><\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[405],"tags":[],"class_list":["post-4175","post","type-post","status-publish","format-standard","hentry","category-champ-neuro-sla"],"_links":{"self":[{"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/posts\/4175","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=4175"}],"version-history":[{"count":19,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/posts\/4175\/revisions"}],"predecessor-version":[{"id":7753,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=\/wp\/v2\/posts\/4175\/revisions\/7753"}],"wp:attachment":[{"href":"https:\/\/kineaphp.fr\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=4175"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=4175"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/kineaphp.fr\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=4175"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}