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NEUROLOGY 2005;64:1261-1262
© 2005 American Academy of Neurology
Brief Communications

Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale

S. H. Subramony, MD, W. May, PhD, D. Lynch, MD, PhD, C. Gomez, MD, PhD, K. Fischbeck, MD, M. Hallett, MD, P. Taylor, MD, R. Wilson, MD, PhD, T. Ashizawa, MD for the Cooperative Ataxia Group

From the Departments of Neurology (Dr. Subramony) and Preventive Medicine (Dr. May), University of Mississippi Medical Center, Jackson; Department of Neurology (Dr. Lynch), Children’s Hospital of Pennsylvania, Philadelphia; Department of Neurology (Dr. Gomez), University of Minnesota, Minneapolis; Neurogenetics Branch (Dr. Fischbeck), NINDS (Dr. Hallett), NIH, Bethesda, MD; Departments of Neurology (Dr. Taylor) and Pathology (Dr. Wilson), University of Pennsylvania, Philadelphia; Department of Neurology (Dr. Ashizawa), University of Texas Medical Branch, Galveston.

Address correspondence and reprint requests to Dr. S.H. Subramony, Department of Neurology, University of Mississippi Medical Center, 2500 N. State Street, Jackson, MS 39216; e-mail: ssubramony{at}neurology.umsmed.edu

Measuring the severity of neurologic dysfunction in patients with inherited ataxias, including Friedreich ataxia (FA), is difficult because of the variable rate of progression, the variable age at onset and the variety of neural systems that may be affected. The authors discuss the problems related to rating scales in the ataxias, report a neurologic rating scale for FA, and demonstrate acceptable interrater reliability of the instrument.

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 April 12 issue to find the title link for this article.

Supported by Friedreich’s Ataxia Research Alliance (FARA).

Received May 3, 2004. Accepted in final form December 7, 2004.




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