Journal article
Health related quality of life measures in Friedreich Ataxia
Journal of the neurological sciences, Vol.272(1), pp.123-128
2008
DOI: 10.1016/j.jns.2008.05.009
PMID: 18571673
Abstract
Evaluation of therapeutic agents for Friedreich Ataxia (FA) has been limited by a lack of adequate markers of disease progression. We assessed the capacity of health related quality of life (HRQOL) questionnaires to reflect disease status in FA. The SF-36 and several symptom-specific scales were administered to an FA cohort. Scores were compared with norms for the United States population, and to a disease-free control group of similar age and gender. FA patients had significantly lower SF-36 Physical Component Summary scores (PCS) and Physical Functioning Subscale (PFS) scores, and both PCS and PFS scores correlated significantly with disease duration and disability status. Mental Component Summary scores (MCS) did not differ between FA patients and controls. Among symptom-specific scales, scores for the Pain Effects, Bladder Control, and Modified Fatigue Impact scales were significantly worse among FA patients than controls, and generally correlated with markers of disease progression. Findings of this study are consistent with the phenotypic characteristics of FA, and suggest that HRQOL measures are potentially useful as clinical markers of disease status in FA.
Details
- Title: Subtitle
- Health related quality of life measures in Friedreich Ataxia
- Creators
- Elizabeth Epstein - Department of Neurology, University of Pennsylvania School of Medicine, USAJennifer M Farmer - Department of Neurology, University of Pennsylvania School of Medicine, USAAmy Tsou - Department of Neurology, University of Pennsylvania School of Medicine, USASusan Perlman - University of California Los Angeles, Los Angeles, CA, USAS.H Subramony - University of Mississippi Medical Center, Jackson, MS, USAChristopher M Gomez - University of Chicago, Chicago IL, USATetsuo Ashizawa - University of Texas Medical Branch, Galveston, TX, USAGeorge R Wilmot - Emory University, Atlanta, GA, USAKatherine Mathews - University of Iowa, Iowa City, IA, USARobert B Wilson - Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, USALaura J Balcer - Department of Neurology, University of Pennsylvania School of Medicine, USADavid R Lynch - Department of Neurology, University of Pennsylvania School of Medicine, USA
- Resource Type
- Journal article
- Publication Details
- Journal of the neurological sciences, Vol.272(1), pp.123-128
- Publisher
- Elsevier B.V
- DOI
- 10.1016/j.jns.2008.05.009
- PMID
- 18571673
- ISSN
- 0022-510X
- eISSN
- 1878-5883
- Language
- English
- Date published
- 2008
- Academic Unit
- Neurology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Neurology (Pediatrics)
- Record Identifier
- 9984020787702771
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