1. Behavioural impairment and frontotemporal dementia in oculopharyngeal muscular dystrophy
- Author
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Baziel G.M. van Engelen, Nicol C. Voermans, Joost Raaphorst, Corinne G.C. Horlings, Maurits Tankink, Barbara M. van der Sluijs, Roy P. C. Kessels, Neurology, ANS - Neurodegeneration, ANS - Neuroinfection & -inflammation, and EURO-NMD
- Subjects
Adult ,Male ,Pediatrics ,medicine.medical_specialty ,Disease duration ,Behavioral Symptoms ,neuromuscular disorders ,Hospital Anxiety and Depression Scale ,Neuropsychiatry ,Motor symptoms ,frontotemporal dementia ,Oculopharyngeal muscular dystrophy ,03 medical and health sciences ,0302 clinical medicine ,All institutes and research themes of the Radboud University Medical Center ,Muscular Dystrophy, Oculopharyngeal ,mental disorders ,Prevalence ,medicine ,Humans ,neuropsychiatry ,030212 general & internal medicine ,Neuro- en revalidatiepsychologie ,business.industry ,behavioural impairment ,Neuropsychology and rehabilitation psychology ,Patient Acuity ,Middle Aged ,medicine.disease ,Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3] ,Neurology ,Health survey ,Female ,Neurology (clinical) ,business ,030217 neurology & neurosurgery ,Frontotemporal dementia - Abstract
Contains fulltext : 236035.pdf (Publisher’s version ) (Open Access) 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. 7 p.
- Published
- 2022
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