Open AccessBehavioural Impairment and Frontotemporal Dementia in Oculopharyngeal Muscular Dystrophy
Author(s) -
Maurits Tankink,
Corinne G.C. Horlings,
Nicol C. Voermans,
Barbara van der Sluijs,
Roy P. C. Kessels,
B.G.M. van Engelen,
Joost Raaphorst
Publication year - 2022
Publication title -
journal of neuromuscular diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.366
H-Index - 23
eISSN - 2214-3602
pISSN - 2214-3599
DOI - 10.3233/jnd-200592
Subject(s) - oculopharyngeal muscular dystrophy , frontotemporal dementia , medicine , depression (economics) , anxiety , dementia , muscular dystrophy , disease , psychiatry , clinical psychology , economics , macroeconomics
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.