Demenza a corpi di Lewy (DLB)
Table of Contents
GENERALITA’
La demenza con corpi di Lewy è una malattia neurodegenerativa progressiva considerata la seconda forma di demenza degenerativa dopo l’Alzheimer e connessa con il Morbo di Parkinson. La DLB è caratterizzata dall’accumulo all’interno delle cellule neuronali e gliali di aggregati insolubili di alfa-sinucleina denominati corpi di Lewy e neuriti di Lewy.
La malattia a corpi di Lewy è caratterizzata da sintomatologia motoria di tipo parkinsoniano associata, fin dalle prime fasi della malattia, a disturbi di tipo cognitivo quali:
- alterazioni della memoria
- allucinazioni
- confusione mentale
Le alterazioni cognitive sono tipicamente fluttuanti per cui un paziente può essere in grado di sostenere lucidamente una conversazione un giorno e mostrarsi confuso, disorientato e incapace di comunicare il giorno successivo.
DIAGNOSI
I criteri per la diagnosi comprendono una combinazione di:
- allucinazioni visive
- fluttuazioni nelle capacità cognitive(memoria, capacità di critica e giudizio)
- sintomi spontanee di parkinsonismo.
Probabilmente la sintomatologia motoria include:
- lentezza dei movimenti
- rigidità e alterazioni posturali,generalmente meno severi che nella malattia di parkinson.
La diagnosi di demenza rappresenta la caratteristica centrale, essenziale per la diagnosi di DLB probabile
o possibile. La fluttuazione dell’attenzione, le allucinazioni visive e il parkinsonismo appartengono al nucleo delle caratteristiche sufficienti per la diagnosi di DLB; se, oltre alla demenza,sono presenti due caratteristiche su tre è legittimo porre la diagnosi di DLB probabile, mentre se è presente una sola caratteristica si pone il sospetto di DLB possibile. La principale diagnosi differenziale si focalizzasulle seguenti patologie:
- le demenze degenerative di tipo corticale, in particolar modo AD;
- la demenza vascolare;
- le sindromi parkinsoniane atipichecomePDD, degenerazione cortico-basale (CBD),
paralisi sopranucleare progressiva (PSP) e atrofia multisistemica MSA; - la malattia di Creutzfeldt-Jakob(CJD).
TERAPIA
La demenza a corpi di Lewy è una malattia degenerativa e inguaribile. Vengono utilizzati i farmaci tipici per la malattia di Parkinson, gli antipsicotici, gli antidepressivi e quelli usati per la malattia di Alzheimer.L’aspettativa di vita stimata per i pazienti affetti da demenza a corpi di Lewy è di circa 8 anni dall’esordio.
ARTICOLI CORRELATI:
Morbo di Parkinson: sintomi, diagnosi e terapia
Levodopa: sindrome da trattamento cronico
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