{"id":5714,"date":"2021-06-11T14:32:56","date_gmt":"2021-06-11T14:32:56","guid":{"rendered":"https:\/\/turkeyistanbulmedical.com\/?p=5714"},"modified":"2022-10-01T13:26:12","modified_gmt":"2022-10-01T13:26:12","slug":"syndrome-dohtahara","status":"publish","type":"post","link":"https:\/\/turkeyistanbulmedical.com\/fr\/syndrome-dohtahara\/","title":{"rendered":"Syndrome d’Ohtahara"},"content":{"rendered":"

Syndrome d’Ohtahara<\/strong><\/h2>\n

Le syndrome d’Ohtahara, cette maladie, qui fait partie des enc\u00e9phalopathies \u00e9pileptiques s\u00e9v\u00e8res du nouveau-n\u00e9, a \u00e9t\u00e9 d\u00e9crite pour la premi\u00e8re fois par Ohtahara en 1976.<\/p>\n

Elle est \u00e9galement connue sous le nom d’enc\u00e9phalopathie \u00e9pileptique infantile pr\u00e9coce. Elle survient dans les 3 premiers mois de la petite enfance.<\/p>\n

Cependant, comme il commence g\u00e9n\u00e9ralement \u00e0 0-1 mois, qui est la p\u00e9riode n\u00e9onatale, il est consid\u00e9r\u00e9 comme faisant partie des syndromes \u00e9pileptiques de cette p\u00e9riode.<\/p>\n

\u00a0<\/strong><\/p>\n

Comment d\u00e9tecter le Syndrome d’Ohtahara<\/a> ?<\/strong><\/h3>\n

La maladie d\u00e9bute par des contractions toniques successives au cours du premier trimestre du b\u00e9b\u00e9.<\/p>\n

Ces contractions toniques sont observ\u00e9es sous forme de mouvements fl\u00e9chisseurs ou extenseurs durant 1 \u00e0 10 secondes. Le nombre de crises par jour de ces contractions peut d\u00e9passer 100. Elle peut \u00e9voluer sous forme de crises cloniques focales dans un tiers des cas.<\/p>\n

Des pointes-ondes multifocales \u00e0 haute tension intenses d’une dur\u00e9e de 2 \u00e0 6 secondes en \u00e9lectroenc\u00e9phalographie sont d\u00e9tect\u00e9es par suppression de tension (B\u00f6rst-Suppression) pendant 3 \u00e0 5 secondes.<\/p>\n

Des anomalies structurelles c\u00e9r\u00e9brales sont souvent responsables de l’\u00e9tiologie.<\/p>\n

Les spasmes peuvent survenir \u00e0 la fois endormis et \u00e9veill\u00e9s. Des crises partielles peuvent survenir juste avant ou apr\u00e8s les spasmes, ou ensemble.<\/p>\n

 <\/p>\n

Quelles sont les causes du Syndrome d’Ohtahara ?<\/strong><\/h3>\n

Bien que la cause exacte ne soit pas connue, on pense qu’elle est probablement due \u00e0 des malformations, des troubles m\u00e9taboliques et certaines mutations g\u00e9n\u00e9tiques.<\/p>\n

Chez la plupart des patients, les effets du syndrome d’Ohtahara peuvent \u00eatre observ\u00e9s dans tous ou certains lobes du cerveau.<\/p>\n

Les effets sur la fonction c\u00e9r\u00e9brale varient selon la fa\u00e7on dont les mutations g\u00e9n\u00e9tiques affectent le d\u00e9veloppement ou la fonction c\u00e9r\u00e9brale.<\/p>\n

 <\/p>\n

A quelles maladies peut-il \u00eatre associ\u00e9 ?<\/strong><\/h3>\n

Un certain nombre de maladies, telles que le syndrome d’Aicardi, la dysplasie d’Oliver Dentat, l’h\u00e9mim\u00e9gancephalie, la porenc\u00e9phalie, l’ag\u00e9n\u00e9sie du corps mamilaire, la dysg\u00e9n\u00e9sie c\u00e9r\u00e9brale et les dysplasies corticales, ont \u00e9t\u00e9 associ\u00e9es \u00e0 des maladies qui surviennent chez les nourrissons apr\u00e8s le syndrome d’Ohtahara.<\/p>\n

 <\/p>\n

Existe-t-il un rem\u00e8de contre le Syndrome d’Ohtahara ?<\/strong><\/h3>\n

Les crises dans le syndrome d’Ohtahara sont r\u00e9sistantes au traitement. La r\u00e9ponse \u00e0 l’ACTH, \u00e0 l’acide valpro\u00efque, aux corticost\u00e9ro\u00efdes, aux benzodiaz\u00e9pines et au phosphate de pyridoxal \u00e0 forte dose utilis\u00e9s dans le traitement de la maladie est tr\u00e8s limit\u00e9e.<\/p>\n

L’effet du r\u00e9gime c\u00e9tog\u00e8ne est controvers\u00e9. Surtout dans les cas de dysplasie corticale, le traitement de l’\u00e9pilepsie peut r\u00e9duire le nombre de crises et soutenir positivement le d\u00e9veloppement psychomoteur du b\u00e9b\u00e9.<\/p>\n

Avec l’\u00e2ge, les crises deviennent des spasmes infantiles typiques; Les r\u00e9sultats de l’EEG se transforment en hypsarythmie.<\/p>\n

Bien que le contr\u00f4le des crises puisse \u00eatre atteint chez la moiti\u00e9 des enfants pouvant atteindre l’\u00e2ge scolaire, la plupart d’entre eux pr\u00e9sentent un retard psychomoteur par rapport \u00e0 leurs pairs.<\/p>\n

 <\/p>\n

\u00c0 quoi ressemblent les enfants atteints du Syndrome d’Ohtahara<\/a> ?<\/strong><\/h4>\n

Les enfants atteints du syndrome d’Ohtahara ont aussi souvent d’autres spasmes. Pour cette raison, la plupart des enfants meurent avant d’avoir atteint l’\u00e2ge de 2 ans. Ces enfants deviennent handicap\u00e9s physiques et mentaux.a<\/p>\n","protected":false},"excerpt":{"rendered":"

Syndrome d’Ohtahara Le syndrome d’Ohtahara, cette maladie, qui fait partie des enc\u00e9phalopathies \u00e9pileptiques s\u00e9v\u00e8res du nouveau-n\u00e9, a \u00e9t\u00e9 d\u00e9crite pour la premi\u00e8re fois par Ohtahara en 1976. Elle est \u00e9galement connue sous le nom d’enc\u00e9phalopathie \u00e9pileptique infantile pr\u00e9coce. Elle survient dans les 3 premiers mois de la petite enfance. Cependant, comme il commence g\u00e9n\u00e9ralement \u00e0 […]<\/p>\n","protected":false},"author":3,"featured_media":5705,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":[],"categories":[1218],"tags":[1228],"yoast_head":"\nSyndrome d'Ohtahara - Turkey Istanbul Medical<\/title>\n<meta name=\"description\" content=\"Le syndrome d'Ohtahara, cette maladie, qui fait partie des enc\u00e9phalopathies \u00e9pileptiques s\u00e9v\u00e8res du nouveau-n\u00e9, a \u00e9t\u00e9 d\u00e9crite pour la premi\u00e8re fois par\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/turkeyistanbulmedical.com\/fr\/syndrome-dohtahara\/\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Syndrome d'Ohtahara\" \/>\n<meta property=\"og:description\" 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