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NEUROLOGY 2006;66:1711-1716
© 2006 American Academy of Neurology

Measuring Friedreich ataxia

Complementary features of examination and performance measures

D. R. Lynch, MD, PhD, J. M. Farmer, MS, A. Y. Tsou, MD, S. Perlman, MD, S. H. Subramony, MD, C. M. Gomez, MD, PhD, T. Ashizawa, MD, G. R. Wilmot, MD, PhD, R. B. Wilson, MD, PhD and L. J. Balcer, MD, MSCE

From the Departments of Neurology (D.R.L., A.Y.T., L.J.B.), Pediatrics (D.R.L.), and Pathology and Laboratory Medicine (J.M.F., R.B.W.), University of Pennsylvania School of Medicine, and Children's Hospital of Philadelphia (D.R.L.), PA; University of California, Los Angeles (S.P.); University of Mississippi Medical Center (S.H.S.), Jackson; University of Minnesota (C.M.G.), Minneapolis; University of Texas Medical Branch (T.A.), Galveston; and Emory University (G.R.W.), Atlanta, GA.

Address correspondence and reprint requests to Dr. David R. Lynch, Division of Neurology, Children's Hospital of Philadelphia, 502 Abramson Building, Philadelphia, PA 19104-4318; e-mail: lynch{at}pharm.med.upenn.edu

Objective: To examine the potential validity of performance measures and examination-based scales in Friedreich ataxia (FA) by examining their correlation with disease characteristics.

Methods: The authors assessed the properties of a candidate clinical outcome measure, the Friedreich Ataxia Rating Scale (FARS), and simple performance measures (9-hole peg test, the timed 25-foot walk, PATA test, and low-contrast letter acuity) in 155 patients with FA from six institutions, and correlated the scores with disease duration, functional disability, activity of daily living scores, age, and shorter GAA repeat length to assess whether these measures capture the severity of neurologic dysfunction in FA.

Results: Scores for the FARS and performance measures correlated significantly with functional disability, activities of daily living scores, and disease duration, showing that these measures meet essential criteria for construct validity for measuring the progressive nature of FA. In addition, the FARS and transformed performance measures scores were predicted by age and shorter GAA repeat length in linear regression models accounting for sex and testing site. Correlations between performance measures were moderate in magnitude, suggesting that each test captures separate yet related dimensions of neurologic function in FA and that a composite measure might better predict disease status. Composite measures created using cohort means and standard deviations predicted disease status better than or equal to single performance measures or examination-based measures.

Conclusions: The Friedreich Ataxia Rating Scale, performance measures, and performance measure composites provide valid assessments of disease progression in Friedreich ataxia.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the June 13 issue to find the title link for this article.

See also page 1717

Supported by grants from the Muscular Dystrophy Association, the Friedreich Ataxia Research Alliance, and a Beeson Scholar Award from AFAR to D.R.L., and NIH grant EY 13273 to L.J.B.

Disclosure: The authors report no conflicts of interest.

Received August 17, 2005. Accepted in final form February 21, 2006.


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