Panencefalite sclerosante subacuta (PESS)
Table of Contents
Che cos’è la panencefalite sclerosante subacuta?
La panencefalite sclerosante subacuta è una malattia molto rara e letale causata dalla persistenza nel SNC del virus delmorbillo. Si presenta in un caso su 25.000 infezioni, colpisce più frequentemente bambini di età compresa tra i 5 e i 15 anni, ma oltre il 50% dei bimbi affetti ha contratto il morbillo nei primi due anni di vita. Alla base di questo fenomeno vi sarebbero un’alterata risposta cellulomediata da parte dell’ospite durante l’infezione acuta, probabilmente favorita dalla presenza di polimorfismi genetici, che impedirebbe l’eradicazione dell’infezione favorendo e la persistenza nelle SNC.
Quali sono i sintomi della panencefalite sclerosante subacuta?
L’esordio clinico è insidioso con alterazioni del comportamento. Nelle settimane o nei mesi successivi subentra un declino intellettuale con episodi di alterazione della funzionalità motoria e crisi miocloniche e altri sintomi neurologici focali.
L’evoluzione prosegue con la comparsa di:
- Stato stuporoso
- Coreoatetosi(sindrome neurologica caratterizzata dalla presenza di movimenti involontari, incontrollabili ed afinalistici in vari settori del corpo)
- Alterazioni del sistema nervoso autonomofino alla perdita completa delle funzioni corticali
- Coma.
- La mortesopraggiunge in media nel giro di 1-3 anni, anche se nel 20% dei casi la sopravvivenza può essere più lunga (4-14 anni) con episodi di trasmissione apparente e di esacerbazione.
Diagnosi
La diagnosi si basa sull’anamesi, sugli esami clinici, sulle caratteristiche alterazioni EEG grafiche e sulla presenza di anticorpi antimorbillo nel liquido cerebrospinale. La RM evidenzia alterazioni della sostanza bianca periventricolare e sottocorticale, non assumenti il contrasto, associata ad atrofia corticale diffusa. La diagnosi viene posta in base al quadro e ECG e liquorale.
Terapia
Al momento, non è disponibile una terapia curativa per la PESS. L’immunizzazione contro il morbillo è l’unico tipo di prevenzione possibile. Alcuni pazienti rispondono ai farmaci antivirali (ribavirina, amantadina) e necessitano di questo trattamento per il resto della loro vita. Inoltre se la terapia viene sospesa la malattia riparte. Si tratta quindi di terapie che possono arrestare l’evoluzione, ma non guarire il male.
Ti potrebbero interessare:
- Quando si parla di vaccinazione, i genitori non sono responsabili solo per i propri figli – la loro decisione pro o contro la vaccinazione può avere un impatto significativo anche sugli altri! Ci sono casi comprovati di bambini che sono stati infettati dal morbillo mentre erano seduti nella sala d’attesa di un pediatra. Non è cinico questo fatto? Uno di questi bambini è morto un anno fa, affetto da Pess.(Toccante lettera di un padre ..LEGGI)
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