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Title: A longitudinal study of the Friedreich Ataxia Impact Scale.
Austin Authors: Tai, Geneieve;Yiu, Eppie M;Corben, Louise A;Delatycki, Martin B 
Affiliation: Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Victoria, Australia
Department of Paediatrics, University of Melbourne, Parkville, Victoria 3052, Australia
Department of Neurology, Royal Children's Hospital, Parkville, Victoria 3052, Australia
School of Psychological Science, Faculty of Medicine, Nursing and Health Sciences, Monash University, Clayton, Victoria 3168, Australia
Department of Clinical Genetics, Austin Health, Heidelberg, Victoria, Australia
Issue Date: 21-Mar-2015
Publication information: Journal of the Neurological Sciences 2015; 352(1-2): 53-7
Abstract: Quality of life in Friedreich ataxia (FRDA) has been explored using various generic health status measurement tools, most commonly the Short Form Health Survey Version 2 (SF-36v2). The tool did not address many specific issues related to disease impact in people with FRDA. The Friedreich Ataxia Impact Scale (FAIS) was developed to examine clinically relevant areas in FRDA. The aims of the current study were to assess the relationship between the FAIS and clinical characteristics of FRDA, as well as to determine the responsiveness of the FAIS to change over one and two years.One hundred and four individuals with FRDA, homozygous for the GAA expansion in intron 1 of FXN, completed the FAIS at baseline. Seventy individuals completed the FAIS again 12months later and 49 completed the FAIS at 24months. Clinical parameters and neurologic scales (Friedreich Ataxia Rating Scale (FARS)) were also recorded.The total FARS score, onset age and disease duration correlated significantly with FAIS subscales measuring symptoms and physical functioning. The physical and mental summary measures of the SF-36 V2 also correlated well with the FAIS subscales. Speech was the only subscale that demonstrated significant change over one and two years.The FAIS provides valuable insight into the perspective of individuals with FRDA on their health status, and is an important measure of morbidity. It has, however, limited responsiveness to change and its use in intervention studies is questionable.
Gov't Doc #: 25840637
DOI: 10.1016/j.jns.2015.03.024
Type: Journal Article
Subjects: Friedreich Ataxia Impact Scale
Friedreich ataxia
Health status
Rating scales
Appears in Collections:Journal articles

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