Encefalomielite acuta disseminata (ADEM)
Table of Contents
Definizione
L’encefalomielite acuta disseminataè un’ encefalite acuta demielinizzante monofasica che si manifesta temporalmente dopo unavaccinazioneo unamalattia infettiva di natura virale.Insorgenza del quadro clinico a distanza di 10-14 giorni da una vaccinazione (antivaiolosa, antirabbica) o da una malattia virale esantematica (morbillo,varicella,rosoliao di altra natura (influenza,mononucleosi infettiva,parotite). Il morbillo è la malattia più frequentemente associata alla comparsa diencefalomielite acuta disseminataseguito dalla varicella.
Encefalomielite acuta disseminata: patogenesi
Si ritiene che la risposta immunitaria nei confronti dell’agente infettivo o del vaccino inneschi un processo infiammatorio che provoca demielinizzazione; autoanticorpi diretti contro la proteina basica della mielina o altri antigeni della mielina sono stati identificati nel liquido cefalorachidiano di pazienti affetti da ADEM.
Come si manifesta l’encefalomielite acuta disseminata?
Le manifestazioni cliniche compaiono dopo circa 10-14 giorni da una vaccinazione a evoluzione apparentemente normale o dopo una malattia virale che sembrava in fase di risoluzione. In alcuni casi l’esordio è brusco con rapida progressione (da ore a giorni) delle alterazioni neurologiche. Si assiste alla ricomparsa (nelle forme post-infettive) difebbre elevata con cefalea e confusione mentale, letargia sino al coma.Le convulsioni sono frequenti, i segni di malattia neurologica disseminata (emiparesi o quadriparesi, interessamento del tronco cerebrale, cecità corticale, ipereflessia o areflessia) sono costantemente presenti. Nelle forme associate alla varicella è frequente il coinvolgimento cerebellare.
Diagnosi
La diagnosi si basa su criteri epidemiologici precedente vaccinazione o malattia virale, clinici o neuroradiologici. La diagnosi viene confermata dalla risonanza magnetica nucleare e dall’esame del liquido cefalorachidiano. La RMN dell’encefalo mostra tipicamente numerose aree di iperintensità del segnale a carico della sostanza bianca edenhancementdopo somministrazione di gadolino. All’esame del liquido cefalorachidiano si osserva in genere pleiocitosi linfocitaria e un modesto incremento dellaprotidorrachia.
Terapia dell’encefalomielite acuta disseminata
Il trattamento iniziale prevede la somministrazione di alte dosi di steroidi per almeno 5 giorni; nei casi non responsivi si può considerare l’impiego diplasmaferesi o di immunoglobuline per via parentale.
Prognosi
La prognosi è gravata da un elevata letalità 10-20% nelle forme postinfettive e da frequenti e importanti sequele neurologiche in coloro che sopravvivono.
Articoli correlati:
(Bibl: manueale di malattie infettive; Moroni, Spinello Vullo)
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