TY - JOUR
T1 - Behavioural Impairment and Frontotemporal Dementia in Oculopharyngeal Muscular Dystrophy
AU - Tankink, Maurits
AU - Horlings, Corinne G. C.
AU - Voermans, Nicol
AU - van der Sluijs, Barbara
AU - Kessels, Roy P. C.
AU - van Engelen, Baziel
AU - Raaphorst, Joost
N1 - Publisher Copyright: © 2022 - The authors. Published by IOS Press.
PY - 2022
Y1 - 2022
N2 - 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.
AB - 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.
KW - Oculopharyngeal muscular dystrophy
KW - behavioural impairment
KW - frontotemporal dementia
KW - neuromuscular disorders
KW - neuropsychiatry
UR - http://www.scopus.com/inward/record.url?scp=85122830254&partnerID=8YFLogxK
U2 - https://doi.org/10.3233/JND-200592
DO - https://doi.org/10.3233/JND-200592
M3 - Article
C2 - 34334411
SN - 2214-3599
VL - 9
SP - 129
EP - 135
JO - Journal of Neuromuscular Diseases
JF - Journal of Neuromuscular Diseases
IS - 1
ER -