Sarah Youssof1. 1. Department of Neurology, University of New Mexico Health Sciences Center, MSC 10 5620, Albuquerque, New Mexico, 87131, USA.
Abstract
INTRODUCTION: Oculopharyngeal muscular dystrophy (OPMD) causes ptosis, dysphagia, and limb weakness. Health-related quality of life (HRQoL) and its relationship to physical symptoms was investigated. METHODS: The 36-item Short Form (SF-36) was completed by 89 participants in the U.S. OPMD Registry. Multiple hierarchical regression was used to determine the relative contributions of dysphagia severity and lower extremity functional impairment to the physical (PCS) and mental (MCS) components of the SF-36. RESULTS: HRQoL was reduced in OPMD compared with population norms. Lower extremity functional impairment explained a significant proportion of variance in PCS and MCS. Dysphagia symptom severity explained a moderate amount of variance only in MCS. Dysphagia symptom severity had the strongest associations with general health perception and social functioning domains. CONCLUSIONS: Lower extremity functional impairment in OPMD deserves attention due to its large influence on HRQoL. Both generic and dysphagia-specific measures are necessary to assess HRQoL in OPMD.
INTRODUCTION:Oculopharyngeal muscular dystrophy (OPMD) causes ptosis, dysphagia, and limb weakness. Health-related quality of life (HRQoL) and its relationship to physical symptoms was investigated. METHODS: The 36-item Short Form (SF-36) was completed by 89 participants in the U.S. OPMD Registry. Multiple hierarchical regression was used to determine the relative contributions of dysphagia severity and lower extremity functional impairment to the physical (PCS) and mental (MCS) components of the SF-36. RESULTS: HRQoL was reduced in OPMD compared with population norms. Lower extremity functional impairment explained a significant proportion of variance in PCS and MCS. Dysphagia symptom severity explained a moderate amount of variance only in MCS. Dysphagia symptom severity had the strongest associations with general health perception and social functioning domains. CONCLUSIONS: Lower extremity functional impairment in OPMD deserves attention due to its large influence on HRQoL. Both generic and dysphagia-specific measures are necessary to assess HRQoL in OPMD.
Authors: Michael R Rose; Reza Sadjadi; John Weinman; Tayyaba Akhtar; Shree Pandya; John T Kissel; Carlayne E Jackson Journal: Muscle Nerve Date: 2012-09 Impact factor: 3.217
Authors: Nicoline Voet; Gijs Bleijenberg; Jan Hendriks; Imelda de Groot; George Padberg; Baziel van Engelen; Alexander Geurts Journal: Neurology Date: 2014-10-22 Impact factor: 9.910
Authors: Chad Heatwole; Rita Bode; Nicholas Johnson; Christine Quinn; William Martens; Michael P McDermott; Nan Rothrock; Charles Thornton; Barbara Vickrey; David Victorson; Richard Moxley Journal: Neurology Date: 2012-07-11 Impact factor: 9.910
Authors: Christopher D Graham; John Weinman; Reza Sadjadi; Trudie Chalder; Richard Petty; Mike G Hanna; Chris Turner; Matt Parton; Paul Maddison; Aleksandar Radunovic; Cheryl Longman; Yvonne Robb; Kate Bushby; David Hilton-Jones; Michael R Rose Journal: Clin Rehabil Date: 2013-11-15 Impact factor: 3.477
Authors: Katherine E Vest; Brittany L Phillips; Ayan Banerjee; Luciano H Apponi; Eric B Dammer; Weiting Xu; Dinghai Zheng; Julia Yu; Bin Tian; Grace K Pavlath; Anita H Corbett Journal: Hum Mol Genet Date: 2017-09-01 Impact factor: 6.150