4. A Rett-szindróma klinikai lefolyásának szakaszai

III. stádium
Stagnálás
Életkor: óvodás – iskolás évek
Időtartam: évek

Ez az állapot 2 és 10 éves kor között a gyors leromlás időszakát követi. Az apraxia, a motoros (mozgási) problémák és a görcsök egyre inkább előtérbe kerülnek. Javulás észlelhető azonban a magatartásban, enyhül az ingerlékenység, kevesebb sírással és kevesebb autisztikus vonással találkozunk. A kislány több érdeklődést mutat környezete iránt, ébersége, figyelmi köre és kommunikációs készségei javulnak. Sok RSZ-ás lány életének nagy részében ebben a stádiumban marad.
 
IV. A stádium (korábban járóképes volt)
IV. B stádium (sohasem volt járóképes)
Késői motoros hanyatlás
Életkor: a III. stádium elmúlását követően
Időtartam: évtizedekre terjedhet

Ez a stádium általában a 10. életéve után kezdődhet, és a csökkent mobilitás jellemzi. A lányok egy része abbahagyja a járást, míg mások soha sem tudtak járni. Nincs hanyatlás a gondolkodásban, a kommunikációban vagy a kézügyességben. Az ismétlődő kézmozgások csökkenhetnek. A scoliosis a legjellemzőbb és a leginkább meghatározó tünet. A tekintettel mutatás általában javul. Az izomzatra jellemző a rigiditás (merevség) és a dystonia (fokozott izomtónus abnormális végtag- vagy törzshelyzetekkel). A pubertás a várható életkorban kezdődik a legtöbb lánynál.

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