{"id":5716,"date":"2021-06-11T14:43:46","date_gmt":"2021-06-11T14:43:46","guid":{"rendered":"https:\/\/turkeyistanbulmedical.com\/?p=5716"},"modified":"2021-06-11T14:53:07","modified_gmt":"2021-06-11T14:53:07","slug":"ohtahara-sendromu","status":"publish","type":"post","link":"https:\/\/turkeyistanbulmedical.com\/tr\/ohtahara-sendromu\/","title":{"rendered":"Ohtahara Sendromu"},"content":{"rendered":"

Ohtahara Sendromu<\/strong><\/h2>\n

Ohtahara sendromu<\/a>,<\/strong> Yeni do\u011fanlardaki ciddi epileptik ansefalopati tablolar\u0131ndan biri olan bu hastal\u0131k ilk kez 1976 y\u0131l\u0131nda Ohtahara taraf\u0131ndan tan\u0131mlanm\u0131\u015ft\u0131r. Erken \u0130nfantil Epileptik Ensefalopati olarakda bilinir. Bebekli\u011fin ilk 3 ay\u0131nda ortaya \u00e7\u0131kar. Ancak genellikle yenido\u011fan d\u00f6nemi olan 0-1 ayda ba\u015flad\u0131\u011f\u0131 i\u00e7in o d\u00f6nemin epilepsi sendromlar\u0131 i\u00e7inde ele al\u0131n\u0131r.<\/p>\n

 <\/p>\n

\u00a0Nas\u0131l Tespit Edilebilir?<\/strong><\/h3>\n

Hastal\u0131k bebe\u011fin ilk \u00fc\u00e7 ay\u0131nda ard\u0131\u015f\u0131k tonik kas\u0131lmalarla ba\u015flar. Bu tonik kas\u0131lmalar 1-10 saniye s\u00fcren fleks\u00f6r veya ekstans\u00f6r hareketler olarak g\u00f6zlenir. Bu kas\u0131lmalar\u0131n g\u00fcnl\u00fck n\u00f6bet say\u0131s\u0131 100’\u00fc ge\u00e7ebilir. Olgular\u0131n \u00fc\u00e7te birinde fokal klonik n\u00f6betler \u015feklinde seyredebilir. Elekroansefalografide 2-6 saniye s\u00fcren yo\u011fun y\u00fcksek voltajl\u0131 m\u00fcltifokal diken-dalgalar\u0131, takip eden 3-5 saniye s\u00fcreli voltaj bask\u0131lanmas\u0131 ile (B\u00f6rst-Supresyon) saptan\u0131r. Etyolojiden \u00e7o\u011fu kez yap\u0131sal beyin anomalileri sorumludur. Spazmlar hem uykuda hem de uyan\u0131kken olabilir. Parsiyel n\u00f6betler spazmlardan hemen \u00f6nce veya sonra veya beraber ortaya \u00e7\u0131kabilir.<\/p>\n

 <\/p>\n

Ohtahara Sendromu Neden Olur?<\/strong><\/h3>\n

Nedeni tam olarak bilinmemekle birlikte muhtemelen malformasyonlar, metabolik bozukluklar ve baz\u0131 gen mutasyonlar\u0131 nedeniyle ortaya \u00e7\u0131kt\u0131\u011f\u0131 d\u00fc\u015f\u00fcn\u00fclmektedir. Hastalar\u0131n b\u00fcy\u00fck bir k\u0131sm\u0131nda beynin t\u00fcm loblar\u0131nda veya baz\u0131 loblar\u0131nda\u00a0Ohtahara Sendromunun<\/strong>\u00a0etkileri g\u00f6zlenebilir.<\/p>\n

Gen mutasyonlar\u0131, genin beyin geli\u015fimini veya i\u015flevini nas\u0131l etkiledi\u011fine ba\u011fl\u0131 olarak beyin fonksiyonlar\u0131ndaki etkiler de\u011fi\u015fkenlik g\u00f6stermektedir.<\/p>\n

 <\/p>\n

Hangi Hastal\u0131klarla \u0130li\u015fkilendirilebilir?<\/strong><\/h3>\n

Aicardi sendromu, OliverDentat displazisi, Hemimegansefali, poransefali, mamilar cisim agenezisi, serebral disgenezi ve kortikal displaziler gibi bir dizi hastal\u0131k,Ohtahara Sendromu<\/strong>\u00a0sonras\u0131 bebeklerde ba\u015f g\u00f6steren hastal\u0131klar olarak ili\u015fkilendirilmi\u015ftir.<\/p>\n

 <\/p>\n

Tedavisi Var m\u0131?<\/strong><\/h4>\n

Ohtahara sendromunda<\/strong>\u00a0n\u00f6betler tedaviye diren\u00e7 g\u00f6sterir. Hastal\u0131\u011f\u0131n tedavi s\u0131ras\u0131nda tedavide kullan\u0131lan ACTH, valproik asit, kortikostroidler, benzodiyazepinler ve y\u00fcksek doz pridoksal fosfata cevap \u00e7ok s\u0131n\u0131rl\u0131d\u0131r. Ketojenik diyetin etkisi ise tart\u0131\u015fmal\u0131d\u0131r. \u00d6zellikle kortikal dizplazili durumlarda epilepsi tedavisi n\u00f6bet say\u0131s\u0131n\u0131 d\u00fc\u015f\u00fcrebilmekte ve bebe\u011fin psikomotor geli\u015fimini olumlu y\u00f6nde desteklemektedir. Ya\u015f art\u0131\u015f\u0131 ile birlikte n\u00f6betler tipik infantil spazmlara; EEG bulgular\u0131 ise hipsaritmiye d\u00f6n\u00fc\u015f\u00fcr. Okul \u00e7a\u011f\u0131na gelebilen \u00e7ocuk hastalar\u0131n yar\u0131s\u0131nda n\u00f6bet kontrol\u00fc sa\u011flanabilmesine kar\u015f\u0131n, \u00e7o\u011funda ya\u015f\u0131tlara nazaran psikomotor gerilik g\u00f6zlenir.<\/p>\n

 <\/p>\n

Ohtahara Sendromu Olan \u00c7ocuklar Nas\u0131l G\u00f6r\u00fcn\u00fcr?<\/strong><\/h4>\n

Ohtahara Sendromu<\/strong><\/a>\u00a0olan \u00e7ocuklarda genellikle di\u011fer spazmlar da ba\u015f g\u00f6sterir. Bu nedenle \u00e7ocuklar\u0131n b\u00fcy\u00fck bir k\u0131sm\u0131 2 ya\u015f\u0131na gelmeden \u00f6l\u00fcr. Bu \u00e7ocuklar fiziksel ve zihinsel engelli olurlar.<\/p>\n","protected":false},"excerpt":{"rendered":"

Ohtahara Sendromu Ohtahara sendromu, Yeni do\u011fanlardaki ciddi epileptik ansefalopati tablolar\u0131ndan biri olan bu hastal\u0131k ilk kez 1976 y\u0131l\u0131nda Ohtahara taraf\u0131ndan tan\u0131mlanm\u0131\u015ft\u0131r. Erken \u0130nfantil Epileptik Ensefalopati olarakda bilinir. Bebekli\u011fin ilk 3 ay\u0131nda ortaya \u00e7\u0131kar. Ancak genellikle yenido\u011fan d\u00f6nemi olan 0-1 ayda ba\u015flad\u0131\u011f\u0131 i\u00e7in o d\u00f6nemin epilepsi sendromlar\u0131 i\u00e7inde ele al\u0131n\u0131r.   \u00a0Nas\u0131l Tespit Edilebilir? Hastal\u0131k bebe\u011fin […]<\/p>\n","protected":false},"author":3,"featured_media":5706,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":[],"categories":[1215],"tags":[1229],"yoast_head":"\nOhtahara Sendromu - Turkey Istanbul Medical<\/title>\n<meta name=\"description\" content=\"Ohtahara sendromu, Yeni do\u011fanlardaki ciddi epileptik ansefalopati tablolar\u0131ndan biri olan bu hastal\u0131k ilk kez 1976 y\u0131l\u0131nda Ohtahara taraf\u0131ndan\" \/>\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\/tr\/ohtahara-sendromu\/\" \/>\n<meta property=\"og:locale\" content=\"tr_TR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Ohtahara Sendromu\" \/>\n<meta property=\"og:description\" content=\"Ohtahara sendromu, Yeni do\u011fanlardaki ciddi 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