West Sendromu
Özet
West sendromu; infantil spazmlar, EEG'de hipsaritmi ve gelişimsel gerileme ile karakterize, genellikle iki yaş altı çocuklarda görülen epileptik bir sendromdur. Genetik mutasyonlar, yapısal beyin anomalileri ve metabolik hastalıklar gibi çeşitli etiyolojik faktörler hastalığın gelişiminde rol oynar. Erken tanı ve ACTH, vigabatrin veya kortikosteroid gibi tedaviler, prognozun iyileştirilmesinde altın standart kabul edilir. Hastalığın seyri ve uzun vadeli sonuçları, altta yatan nedene ve tedaviye başlama hızına göre büyük ölçüde değişkenlik gösterir.
West syndrome is an epileptic condition characterized by infantile spasms, EEG hypsarrhythmia, and developmental regression, typically emerging before two years of age. Pathogenesis involves various structural, genetic, metabolic, and immunologic factors, although a significant proportion of cases lack a clear etiology. Immediate diagnosis and administration of treatments such as ACTH, vigabatrin, or corticosteroids are critical for management. Prognosis is highly heterogeneous, depending primarily on the underlying cause and the timeliness of clinical intervention.
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