Behavioural Impairment and Frontotemporal Dementia in Oculopharyngeal Muscular Dystrophy

Some patients with Oculopharyngeal Muscular Dystrophy (OPMD) develop frontotemporal dementia (FTD). The prevalence and clinical correlates of behavioural impairment, including FTD, is unknown in OPMD. 24 OPMD patients and their proxies completed a questionnaire concerning behavioural impairment (ALS...

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Detalles Bibliográficos
Autores principales: Tankink, Maurits, Horlings, Corinne G.C., Voermans, Nicol, van der Sluijs, Barbara, Kessels, Roy P.C., van Engelen, Baziel, Raaphorst, Joost
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2022
Materias:
Short Commentary
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8842748/
https://www.ncbi.nlm.nih.gov/pubmed/34334411
http://dx.doi.org/10.3233/JND-200592
Descripción
Sumario:Some patients with Oculopharyngeal Muscular Dystrophy (OPMD) develop frontotemporal dementia (FTD). The prevalence and clinical correlates of behavioural impairment, including FTD, is unknown in OPMD. 24 OPMD patients and their proxies completed a questionnaire concerning behavioural impairment (ALS-FTD-Q). We examined proportions with mild or severe behavioural changes, according to validated cut-off proxy scores. We examined correlations with the Hospital Anxiety and Depression Scale (HADS), the Short Form Health Survey (SF-36), motor symptoms, genotype and disease duration. In this small patient sample, behavioural impairment was present in 29%of OPMD patients; in 17%the severity of symptoms was compatible with bvFTD. Correlations were small to medium.

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