title: Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale.
abstract: 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.
authors.affiliation: Department of Neurology, University of Mississippi Medical Center, Jackson 39216, USA. ssubramony@neurology.umsmed.edu
authors.forename: S H
authors.initials: SH
authors.lastname: Subramony
authors.forename: W
authors.initials: W
authors.lastname: May
authors.forename: D
authors.initials: D
authors.lastname: Lynch
authors.forename: C
authors.initials: C
authors.lastname: Gomez
authors.forename: K
authors.initials: K
authors.lastname: Fischbeck
authors.forename: M
authors.initials: M
authors.lastname: Hallett
authors.forename: P
authors.initials: P
authors.lastname: Taylor
authors.forename: R
authors.initials: R
authors.lastname: Wilson
authors.forename: T
authors.initials: T
authors.lastname: Ashizawa
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D001132:Arm / Q000503:physiopathology; D001933:Brain Stem / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005621:Friedreich Ataxia / Q000175:diagnosis* / Q000503:physiopathology; D020233:Gait Disorders, Neurologic / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D007866:Leg / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination / Q000592:standards; D015588:Observer Variation; D010525:Peripheral Nerves / Q000503:physiopathology; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D011237:Predictive Value of Tests; D015203:Reproducibility of Results; D012720:Severity of Illness Index