Landau Kleffner syndrome, electrical status epilepticus in sleep and autistic regression: An overview of literature

Çocuklarda dil regresyonu, tanı ve tedavi yaklaşımları ile ilgili yeterli düzeyde araştırma yapılmamış olması nedeniyle, klinisyenler için zorlayıcı bir durumdur. Landau Kleffner sendromu (LKS), uykuda elektriksel status epilepticus (ESES) ve otistik regresyon (AR) nadir görülen ve dil regresyonun eşlik ettiği klinik durumlar arasında yer almaktadır. LKS ve ESES, aynı epileptik sendrom spektrumu içinde yer alan, uykuda aktive olan paroksismal EEG aktivitesi ve regresyonun olduğu iki klinik antite olarak değerlendirilmektedir. Ancak yapılan araştırmalar, AR’nin LKS ve ESES’den birçok açıdan farklılıklar gösterdiğini ortaya koymuştur. Her üç klinik durumda da, prognoz için en önemli faktörler arasında erken tanı ve tedavi kaçınılmaz görülmektedir. Uyku EEG’si LKS ve ESES için önemli bir tanısal değer taşımaktadır. Bu derleme yazısında, LKS, ESES ve AR’nin klinik özellikleri, prognozları ve tedavi yaklaşımları tartışılacaktır.

Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

Language regression in children is a challenging condition for clinicians since the diagnostic and therapeutic algorithms are not well established. Landau Kleffner syndrome (LKS), Electrical Status Epilepticus in Sleep (ESES) and Autistic Regression (AR) are three rare clinical entities in which langauage regression is present. LKS and ESES appear to be two points on the spectrum of epileptic syndromes characterized by severe paroxysmal EEG disturbance in sleep and regression. However, the available literature indicates that AR has many distinct features than LKS and ESES. For all of these three conditions, early recognition and treatment is crucial for favorable outcomes. Overnight EEG has an important diagnostic role for LKS and ESES. In this review, the clinical features, prognosis and treatment options of LKS, ESES and AR will be discussed.

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