Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale

Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale

Subramony S H; May W; Lynch D; Gomez C; Fischbeck K; Hallett M; Taylor P; Wilson R; Ashizawa T; Cooperative Ataxia Group

Neurology

2005

Adolescent; Adult; Age of Onset; Arm/pp [Physiopathology]; Brain Stem/pp [Physiopathology]; Disease Progression; Female; Friedreich Ataxia/di [Diagnosis]; Friedreich Ataxia/pp [Physiopathology]; Gait Disorders; Neurologic/di [Diagnosis]; Gait Disorders; Neurologic/et [Etiology]; Gait Disorders; tone and motor problems; Friedreich's ataxia; tool development; scale development; Friedrich's Ataxia Scale

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.

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