ALS PATOFİZYOLOJİSİ: NEYİ, NE KADAR BİLİYORUZ?

Abstract

Amyotrofik lateral skleroz (ALS) esas olarak serebral korteks, beyin sapı ve spinal korddaki motor nöronları etkileyen progresif seyirli nörodejeneratif bir hastalıktır. Çoğu vakanın sporadik olduğu ALS’de familyal geçiş %5-10’dur ve bunların yaklaşık olarak %10-20’sinde neden süperoksid dismutaz-1 enziminde meydana gelen mutasyondur. Familyal ve sporadik vakaların klinik ve patolojik bulguları birbirlerine büyük benzerlikler göstermektedir. Bu özgün derleme yazısında ALS’nin patofizyolojisinde etkili olduğu öne sürülen mekanizmalar varsayımsal tümleyici bir model ile tartışılacaktır.

Keywords

• ALS, Patofizyoloji, Varsayımsal tümleyici model

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