Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale

Neurology. 2005 Apr 12;64(7):1261-2. doi: 10.1212/01.WNL.0000156802.15466.79.

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Arm / physiopathology
  • Brain Stem / physiopathology
  • Disease Progression
  • Female
  • Friedreich Ataxia / diagnosis*
  • Friedreich Ataxia / physiopathology
  • Gait Disorders, Neurologic / diagnosis
  • Gait Disorders, Neurologic / etiology
  • Gait Disorders, Neurologic / physiopathology
  • Humans
  • Leg / physiopathology
  • Male
  • Middle Aged
  • Neurologic Examination / standards
  • Observer Variation
  • Peripheral Nerves / physiopathology
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / etiology
  • Peripheral Nervous System Diseases / physiopathology
  • Predictive Value of Tests
  • Reproducibility of Results
  • Severity of Illness Index*