Malattia di Huntington: malattia genetica rara
Table of Contents
Che cos’è la malattia di Huntington?
Malattia di Huntington è una malattia degenerativa progressivaa ereditarietà autosomica dominante, caratterizzata daalterazioni della funzione motoria, comportamentale e cognitiva. Insorge tipicamente tra i 25 e i 45 anni e il suo segno patognomonico è la presenza di movimenti coreici involontari rapidi, disordinati e parzialmente finalizzati, spesso associata a disartria, disturbi della marcia e dei movimenti oculari.
Quali sono i sintomi della malattia di Huntington?
Gli stadi più avanzati sono caratterizzati da una riduzione della corea con insorgenza di:
- Distonia
- Rigidità
- Bradicinesia
- Mioclono
- Spasticità
I pazienti con malattia di Huntington sviluppano, nelle fasi terminali, anche disturbi del comportamento e alterazioni cognitive, che possono essere la principali cause di disabilità. E’ una malattia autosomica dominante causata da un’espansione del numero di triplette poliglutamminiche (CAG) nella sequenza codificante del geneHuntingtonsul cromosaoma 4.
Come avviene la diagnosi della malattia di Huntington ?
E’ disponibile il test genetico per diagnosticare la malattia di Huntington.In un soggetto anche privodi sintomi si può effettuare l’indagine genetica di ricerca del gene mutato su un semplice prelievo di sangue che consente di accertare la presenza o l’assenza della mutazione nel gene IT-15. La decisione di sottoporsi al test coinvolge non solo il singolo individuo ma anche la sua famiglia (coniuge, figli, fratelli).
Come può essere curata la malattia di Huntington?
Il trattamento prevede unapproccio multidisciplinare con consulenze neuropsichiatre, sociali e geneticheper i pazienti e le loro famiglie. Gli antidopaminergici possono controllare la corea ma aggravare i sintomi motori, e spesso si associano a un rischio eccessivo di effetti collaterali.La psicosipuò essere trattata con neurolettici atipici. Attualmente non esistono farmaci in grado di modificare la storia naturale della malattia.
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References:
https://2workinoz.com.au/employers/online-options-active-in-australia/
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