{"id":72520,"date":"2019-01-31T13:55:41","date_gmt":"2019-01-31T13:55:41","guid":{"rendered":"http:\/\/humanitasnet-new.local\/malattie\/sclerose-laterale-primitive-sla\/"},"modified":"2025-01-13T03:13:40","modified_gmt":"2025-01-13T03:13:40","slug":"sclerose-laterale-primitive-sla","status":"publish","type":"disease","link":"https:\/\/humanitas-net.humweb.webiz.team\/fr\/diseases\/sclerose-laterale-primitive-sla\/","title":{"rendered":"Scl\u00e9rose lat\u00e9rale primitive (SLA)"},"content":{"rendered":"

\u00a0<\/span>La scl\u00e9rose lat\u00e9rale primitive (SLA), qui appartient au groupe des maladies des motoneurones, est une maladie neuromusculaire rare caract\u00e9ris\u00e9e par une faiblesse musculaire progressive des muscles volontaires, comme ceux que vous utilisez pour contr\u00f4ler les jambes, les bras et la langue. Le SLA n’affecte que les motoneurones sup\u00e9rieurs et c’est un type de maladie des motoneurones qui provoque la lente d\u00e9gradation des cellules nerveuses musculaires, entra\u00eenant une faiblesse.<\/span><\/p>\n

La scl\u00e9rose lat\u00e9rale primitive peut survenir \u00e0 tout \u00e2ge, mais elle survient habituellement entre 40 et 60 ans. La scl\u00e9rose lat\u00e9rale primitive est souvent confondue avec une autre maladie des motoneurones plus courante appel\u00e9e scl\u00e9rose lat\u00e9rale amyotrophique (SLA). Cependant, la scl\u00e9rose lat\u00e9rale primitive progresse plus lentement que la SLA et, dans la plupart des cas, n’est pas mortelle.<\/span><\/p>\n

\u00a0<\/span><\/span><\/p>\n

Sympt\u00f4mes<\/span><\/h2>\n

Les sympt\u00f4mes de la SLA mettent habituellement des ann\u00e9es \u00e0 se manifester et la marque distinctive de la SLA est la faiblesse progressive et la spasticit\u00e9 des muscles volontaires. Les premiers sympt\u00f4mes sont souvent la raideur, la spasticit\u00e9, les tr\u00e9buchements ou la difficult\u00e9 \u00e0 trouver l’\u00e9quilibre et \u00e0 soulever les jambes. D’autres personnes peuvent \u00eatre les premi\u00e8res \u00e0 remarquer un changement dans la d\u00e9marche de la personne atteinte. Quelques autres sympt\u00f4mes qui sont \u00e9prouv\u00e9s par les patients incluent notamment :<\/span><\/p>\n

\u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 <\/span><\/span><\/span>Faiblesse et raideur progressant vers le tronc, puis les bras, les mains, la langue et la m\u00e2choire ;<\/span><\/p>\n

\u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 <\/span><\/span><\/span>L’enrouement, la r\u00e9duction de la vitesse d’\u00e9locution, les troubles de l’\u00e9locution et la bave \u00e0 mesure que les muscles du visage s’affaiblissent ;<\/span><\/p>\n

\u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 <\/span><\/span><\/span>Bave et difficult\u00e9s \u00e0 avaler et \u00e0 respirer tard dans la maladie<\/span><\/p>\n

\u00b7\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0\u00a0 <\/span><\/span><\/span>Occasionnellement, certains patients souffrent de troubles de la parole et de la d\u00e9glutition.<\/span><\/p>\n

\u00a0<\/span><\/span><\/p>\n

Causes<\/span><\/h2>\n

La cause de la scl\u00e9rose lat\u00e9rale primaire chez l’adulte est inconnue. Dans la plupart des cas, il ne s’agit pas d’une maladie h\u00e9r\u00e9ditaire et on ne sait pas pourquoi ni comment elle commence. La scl\u00e9rose lat\u00e9rale primaire juv\u00e9nile, par contre, est une maladie autosomique r\u00e9cessive, ce qui signifie que les deux parents doivent \u00eatre porteurs du g\u00e8ne pour le transmettre \u00e0 leur enfant, m\u00eame s’ils ne sont pas eux-m\u00eames atteints de cette maladie. La SLA juv\u00e9nile est caus\u00e9e par des mutations dans un g\u00e8ne appel\u00e9 ALS2. Bien que les chercheurs ne comprennent pas comment ce g\u00e8ne cause la maladie, ils savent que le g\u00e8ne ALS2 est responsable de la cr\u00e9ation de la prot\u00e9ine alsine, qui est pr\u00e9sente dans les cellules des motoneurones. Lorsque les instructions sont modifi\u00e9es, l’alsine devient instable, ce qui \u00e0 son tour alt\u00e8re la fonction musculaire normale.<\/span><\/p>\n

\u00a0<\/span><\/span><\/p>\n

Complications<\/span><\/h2>\n

Il est impossible de pr\u00e9dire la vitesse \u00e0 laquelle les sympt\u00f4mes \u00e9volueront et leur gravit\u00e9. Bien que la progression moyenne dure environ 20 ans, la maladie a des effets tr\u00e8s variables d’une personne \u00e0 l’autre. Certaines personnes peuvent \u00eatre capables de continuer \u00e0 marcher, mais d’autres devront \u00e9ventuellement utiliser des fauteuils roulants ou d’autres appareils fonctionnels. On ne pense pas que la SLA chez l’adulte raccourcisse l’esp\u00e9rance de vie, mais elle peut avoir une incidence graduelle sur la qualit\u00e9 de vie \u00e0 mesure que de plus en plus de muscles deviennent handicap\u00e9s. Des muscles plus faibles peuvent vous faire tomber davantage, ce qui peut entra\u00eener des blessures.<\/span><\/p>\n","protected":false},"featured_media":0,"template":"","class_list":["post-72520","disease","type-disease","status-publish","hentry"],"yoast_head":"\nScl\u00e9rose lat\u00e9rale primitive (SLA) - Humanitas.net<\/title>\n<meta name=\"robots\" content=\"noindex, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Scl\u00e9rose lat\u00e9rale primitive (SLA) - Humanitas.net\" \/>\n<meta property=\"og:description\" content=\"\u00a0La scl\u00e9rose lat\u00e9rale primitive (SLA), qui appartient au groupe des maladies des motoneurones, est une maladie neuromusculaire rare caract\u00e9ris\u00e9e par une faiblesse musculaire progressive des muscles volontaires, comme ceux que vous utilisez pour contr\u00f4ler les jambes, les bras et la langue. 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