Psychometric Properties of the Friedreich Ataxia Rating Scale

Overview

Journal Neurol Genet

Date 2020 Feb 12

PMID 32042904

Citations 35

Authors

Christian Rummey

Louise A Corben

Martin B Delatycki

S H Subramony

Khalaf Bushara

Christopher M Gomez

Joseph Chad Hoyle

Grace Yoon

Bernard Ravina

Katherine D Mathews

George Wilmot

Theresa Zesiewicz

Susan Perlman

Jennifer M Farmer

David R Lynch

Affiliations

Soon will be listed here.

Abstract

Objective: To investigate the psychometric properties of the Friedreich Ataxia Rating Scale neurologic examination (FARSn) and its subscores, as well as the influence of the modifications resulting in the now widely used modified FARS (mFARS) examination.

Methods: Based on cross-sectional FARS data from the FA-Clinical Outcome Measures cohort, we conducted correlation-based psychometric analyses to investigate the interplay of items and subscores within the FARSn/mFARS constructs.

Results: The results provide support for both the FARSn and the mFARS constructs, as well as individually for their upper limb and lower limb coordination components. The omission of the peripheral nervous system subscore (D) and 2 items of the bulbar subscore (A) in the mFARS strengthens the overall construct compared with the complete FARS.

Conclusions: A correlation-based psychometric analysis of the neurologic FARSn score justifies the overall validity of the scale. In addition, omission of items of limited functional significance as created in the mFARS improves the features of the measures. Such information is crucial to the ongoing application of the mFARS in natural history studies and clinical trials. Additional analyses of longitudinal changes will be necessary to fully ascertain its utility, especially in nonambulant patients.

Citing Articles

A global perspective on research advances and future challenges in Friedreich ataxia.

Indelicato E, Delatycki M, Farmer J, Franca Jr M, Perlman S, Rai M Nat Rev Neurol. 2025; .

PMID: 40032987 DOI: 10.1038/s41582-025-01065-y.

Measurement Properties of the Friedreich Ataxia Rating Scale in Patients with Spinocerebellar Ataxia.

Potashman M, Popoff E, Powell L, Beiner M, Mackenzie A, Coric V Neurol Ther. 2025; 14(2):527-545.

PMID: 39806095 PMC: 11906947. DOI: 10.1007/s40120-024-00708-4.

Functional Outcome Measures to Optimize Drug Development in Spinal and Bulbar Muscular Atrophy: Results From a Meta-Analysis of the Global SBMA Dataset.

Huggett S, Tebbenkamp A, Rinaldi C, Jayaseelan D, Zampedri L, Blasi L Neurology. 2024; 103(12):e210088.

PMID: 39591556 PMC: 11666247. DOI: 10.1212/WNL.0000000000210088.

Assessment of the Clinical Interactions of GAA Repeat Expansions in and .

Gerhart B, Pellerin D, Danzi M, Zuchner S, Brais B, Matos-Rodrigues G Neurol Genet. 2024; 10(6):e200210.

PMID: 39574782 PMC: 11581763. DOI: 10.1212/NXG.0000000000200210.

At-home wearable-based monitoring predicts clinical measures and biological biomarkers of disease severity in Friedreich's Ataxia.

Mishra R, Nunes A, Enriquez A, Profeta V, Wells M, Lynch D Commun Med (Lond). 2024; 4(1):217.

PMID: 39468362 PMC: 11519636. DOI: 10.1038/s43856-024-00653-1.

References

Metz G, Coppard N, Cooper J, Delatycki M, Durr A, Di Prospero N . Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database. Brain. 2013; 136(Pt 1):259-68. PMC: 3624678. DOI: 10.1093/brain/aws309. View

Marelli C, Figoni J, Charles P, Anheim M, Tchikviladze M, Vincitorio C . Annual change in Friedreich's ataxia evaluated by the Scale for the Assessment and Rating of Ataxia (SARA) is independent of disease severity. Mov Disord. 2011; 27(1):135-8. DOI: 10.1002/mds.23879. View

Fahey M, Corben L, Collins V, Churchyard A, Delatycki M . How is disease progress in Friedreich's ataxia best measured? A study of four rating scales. J Neurol Neurosurg Psychiatry. 2006; 78(4):411-3. PMC: 2077798. DOI: 10.1136/jnnp.2006.096008. View

Reetz K, Dogan I, Costa A, Dafotakis M, Fedosov K, Giunti P . Biological and clinical characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) cohort: a cross-sectional analysis of baseline data. Lancet Neurol. 2015; 14(2):174-82. DOI: 10.1016/S1474-4422(14)70321-7. View

Reetz K, Dogan I, Hilgers R, Giunti P, Mariotti C, Durr A . Progression characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS): a 2 year cohort study. Lancet Neurol. 2016; 15(13):1346-1354. DOI: 10.1016/S1474-4422(16)30287-3. View

Lynch D, Hauser L, McCormick A, Wells M, Dong Y, McCormack S . Randomized, double-blind, placebo-controlled study of interferon- 1b in Friedreich Ataxia. Ann Clin Transl Neurol. 2019; 6(3):546-553. PMC: 6414489. DOI: 10.1002/acn3.731. View

Koeppen A, Mazurkiewicz J . Friedreich ataxia: neuropathology revised. J Neuropathol Exp Neurol. 2013; 72(2):78-90. PMC: 3817014. DOI: 10.1097/NEN.0b013e31827e5762. View

Milne S, Murphy A, Georgiou-Karistianis N, Yiu E, Delatycki M, Corben L . Psychometric properties of outcome measures evaluating decline in gait in cerebellar ataxia: A systematic review. Gait Posture. 2018; 61:149-162. DOI: 10.1016/j.gaitpost.2017.12.031. View

Subramony S, May W, Lynch D, Gomez C, Fischbeck K, Hallett M . Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale. Neurology. 2005; 64(7):1261-2. DOI: 10.1212/01.WNL.0000156802.15466.79. View

10.

Delatycki M . Evaluating the progression of Friedreich ataxia and its treatment. J Neurol. 2009; 256 Suppl 1:36-41. DOI: 10.1007/s00415-009-1007-y. View

11.

Trouillas P, Takayanagi T, Hallett M, CURRIER R, Subramony S, Wessel K . International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. J Neurol Sci. 1997; 145(2):205-11. DOI: 10.1016/s0022-510x(96)00231-6. View

12.

Patel M, Isaacs C, Seyer L, Brigatti K, Gelbard S, Strawser C . Progression of Friedreich ataxia: quantitative characterization over 5 years. Ann Clin Transl Neurol. 2016; 3(9):684-94. PMC: 5018581. DOI: 10.1002/acn3.332. View

13.

Regner S, Lagedrost S, Plappert T, Paulsen E, Friedman L, Snyder M . Analysis of echocardiograms in a large heterogeneous cohort of patients with friedreich ataxia. Am J Cardiol. 2011; 109(3):401-5. DOI: 10.1016/j.amjcard.2011.09.025. View

14.

Schmitz-Hubsch T, Tezenas Du Montcel S, Baliko L, Berciano J, Boesch S, Depondt C . Scale for the assessment and rating of ataxia: development of a new clinical scale. Neurology. 2006; 66(11):1717-20. DOI: 10.1212/01.wnl.0000219042.60538.92. View

15.

Burk K, Malzig U, Wolf S, Heck S, Dimitriadis K, Schmitz-Hubsch T . Comparison of three clinical rating scales in Friedreich ataxia (FRDA). Mov Disord. 2009; 24(12):1779-84. DOI: 10.1002/mds.22660. View

16.

Tai G, Corben L, Gurrin L, Yiu E, Churchyard A, Fahey M . A study of up to 12 years of follow-up of Friedreich ataxia utilising four measurement tools. J Neurol Neurosurg Psychiatry. 2014; 86(6):660-6. DOI: 10.1136/jnnp-2014-308022. View

17.

Lynch D, Farmer J, Wilson R, Balcer L . Performance measures in Friedreich ataxia: potential utility as clinical outcome tools. Mov Disord. 2005; 20(7):777-82. DOI: 10.1002/mds.20449. View

18.

Di Prospero N, Baker A, Jeffries N, Fischbeck K . Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial. Lancet Neurol. 2007; 6(10):878-86. DOI: 10.1016/S1474-4422(07)70220-X. View

19.

Cano S, Hobart J, Hart P, Korlipara L, Schapira A, Cooper J . International Cooperative Ataxia Rating Scale (ICARS): appropriate for studies of Friedreich's ataxia?. Mov Disord. 2005; 20(12):1585-91. DOI: 10.1002/mds.20651. View

20.

Lynch D, Farmer J, Tsou A, Perlman S, Subramony S, Gomez C . Measuring Friedreich ataxia: complementary features of examination and performance measures. Neurology. 2006; 66(11):1711-6. DOI: 10.1212/01.wnl.0000218155.46739.90. View