Tények röviden a Rett-szindrómáról (RSZ)

  • Neurológiai rendellenesség.
  • Gyakran autizmusként, agyi bénulásként vagy nem specifikus fejlődésbeli elmaradásként diagnosztizálják.
  • Az X kromoszómán található hibás gén okozza.
  • Szinte kizárólag nőknél fordul elő. Szemben a nőkkel, akiknek két X kromoszómájuk van, a férfiak egy X és egy Y kromoszómával rendelkeznek. Mivel a férfiakban az X kromoszómának nincs olyan háttérmásolata, amely kompenzálná a hibásat, az adott gén hibás változatai halálos kimenetelűek a férfi magzatban. Ez az oka annak, hogy a Rett-szindróma szinte kizárólag nőkben fordul elő.
  • Világszerte számos faji és etnikai csoportban előfordul.
  • Előfordulása 1:10 000 – 1:23 000 között mozog, de az előfordulási gyakorisága ettől lényegesen nagyobb is lehet, a feltételezések szerint.
  • Először dr. Andreas Rett írta le 1964-ben.
  • Figyelmet világszerte dr. Bengt Hagberg 1983-ban megjelent első angol nyelvű közlését követően kapott.
  • A fejlődés a 6 – 18. élethónapig normálisnak tűnik.
  • Ezt a megszerzett beszédkészség és a kézügyesség elvesztése, a fej növekedési ütemének lassulása, stereotip, ismétlődő kézmozgások kialakulása, valamint a járóképesség elvesztése vagy annak nehézségei kísérik.
  • A kézmozgások közé tartoznak a kézmosó mozdulatok, a kéz csavarásai, kopogások, csapkodások, illetve a kéz szájba vétele.
  • További problémák közé tartoznak a görcsök, a légzési rendellenességek (hyperventilatio, vagy apnoe), fogcsikorgatás, valamint a gerincferdülés.
  • A RSZ esetek 99,5%-a csak egyszeri előfordulás a családban.

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