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I deficit cognitivi nelle sindromi extrapiramidali con demenza

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Malattia di Parkinson e parkinsonismi

Riassunto

Le malattie cerebrali su base degenerativa che si caratterizzano per il prevalente coinvolgimento del sistema extrapiramidale, sebbene generalmente contraddistinte dalle alterazioni motorie, so no spesso associate a disturbi cognitivi e comportamentali, fino a delinearsi in alcuni casi come vere e proprie sindromi demenziali. Le malattie principali di questo gruppo sono la malattia di Parkinson (MP), la paralisi sopranucleare progressiva (PSP), la malattia di Huntington (HD) e le atrofie multisistemiche che rappresentano forme più rare di disturbi extrapiramidali associati a declino cognitivo. Queste malattie colpiscono principalmente, ma non necessariamente in maniera esclusiva, le strutture sottocorticali dell’encefalo come i gangli della base, il talamo, i nuclei rostrali del troncoencefalo nonché le proiezioni di queste strutture alle aree corticali del lobo frontale e al sistema limbico. In queste patologie tuttavia i disturbi motori sono generalmente preminenti sia in termini di comparsa che di gravità rispetto ai deficit cognitivi che appaiono invece secondari e di accompagnamento alla patologia motoria. Diversamente, nella demenza a corpi di Lewy (DLB), in cui le lesioni neuropatologiche si distribuiscono diffusamente alle strutture sottocorticali e corticali dell’encefalo, i disturbi cognitivi e motori (oltre a peculiari disturbi comportamentali) rappresentano entrambi i sintomi cardinali della patologia (McKeith et al., 2004). Anche nella degenerazione corticobasale (CBD) si assiste all’associazione di una sintomatologia di origine sia corticale che sottocorticale; in questo caso però, il complesso quadro neuropsicologico e le particolari caratteristiche motorie la rendono un’entità clinico-patologica distinta dalle altre malattie dementigene con disturbi motori.

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Perri, R., Carlesimo, G.A. (2009). I deficit cognitivi nelle sindromi extrapiramidali con demenza. In: Malattia di Parkinson e parkinsonismi. Springer, Milano. https://doi.org/10.1007/978-88-470-1490-9_4

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