ArticleBrain : a journal of neurology2013
Rating disease progression of Friedreich's ataxia by the International Cooperative Ataxia Rating Scale: analysis of a 603-patient database.
Article in Brain : a journal of neurology, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 55 citations in OpenAlex.
- Results From a Phase 2, Open-Label Study Evaluating the Safety, Tolerability, and Effect on Ataxia of GLM101 in Three Adult Patients With PMM2-CDG.Journal of inherited metabolic disease · 2026Trial
- Automated functional upper limb evaluation of patients with Friedreich ataxia using serious games rehabilitation exercises.Journal of neuroengineering and rehabilitation · 2018Trial
- An open-label trial in Friedreich ataxia suggests clinical benefit with high-dose resveratrol, without effect on frataxin levels.Journal of neurology · 2015Trial
- Long-term clinical course and extracerebellar manifestations of hereditary spinocerebellar degeneration.Acta neurologica Belgica · 2026Article
- A global perspective on research advances and future challenges in Friedreich ataxia.Nature reviews. Neurology · 2025Review
- Peripheral Inflammation Profile of Cerebellar Ataxia.Current neuropharmacology · 2025Article
- Neurodegenerative Diseases: New Hopes and Perspectives.Current molecular medicine · 2024Review
- Review
- Prediction of the disease course in Friedreich ataxia.Scientific reports · 2022Article
- DNA methylation in Friedreich ataxia silences expression of frataxin isoform E.Scientific reports · 2022Article
- Increased brain tissue sodium concentration in Friedreich ataxia: A multimodal MR imaging study.NeuroImage. Clinical · 2022Article
- Nicotinamide Riboside Improves Ataxia Scores and Immunoglobulin Levels in Ataxia Telangiectasia.Movement disorders : official journal of the Movement Disorder Society · 2021Article
- Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort.Annals of clinical and translational neurology · 2021Observational
- Psychometric properties of the Friedreich Ataxia Rating Scale.Neurology. Genetics · 2019Article
- Evaluation of antibodies for western blot analysis of frataxin protein isoforms.Journal of immunological methods · 2019Article
- Differences in the determinants of right ventricular and regional left ventricular long-axis dysfunction in Friedreich ataxia.PloS one · 2018Article
- Article
- Stratification of disease progression in a broad spectrum of degenerative cerebellar ataxias with a clustering method using MRI-based atrophy rates of brain structures.Cerebellum & ataxias · 2017Article
- Reversal of epigenetic promoter silencing in Friedreich ataxia by a class I histone deacetylase inhibitor.Nucleic acids research · 2016Article
- Translating HDAC inhibitors in Friedreich's ataxia.Expert opinion on orphan drugs · 2016Article
Corrections and comments
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Authors and funding
11 authors at 7 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The aim of this cross-sectional study was to analyse disease progression in Friedreich's ataxia as measured by the International Cooperative Ataxia Rating Scale. Single ratings from 603 patients with Friedreich's ataxia were analysed as a function of disease duration, age of onset and GAA repeat lengths. The relative contribution of items and subscales to the total score was studied as a function of disease progression. In addition, the scaling properties were assessed using standard statistical measures. Average total scale progression per year depends on the age of disease onset, the time since diagnosis and the GAA repeat length. The age of onset inversely correlates with increased GAA repeat length. For patients with an age of onset ≤14 years associated with a longer repeat length, the average yearly rate of decline was 2.5 ± 0.18 points in the total International Cooperative Ataxia Rating Scale for the first 20 years of disease duration, whereas patients with a later onset progress more slowly (1.8 ± 0.27 points/year). Ceiling effects in posture, gait and lower limb scale items lead to a reduced sensitivity of the scale in the severely affected population with a total score of >60 points. Psychometric scaling analysis shows generally favourable properties for the total scale, but the subscale grouping could be improved. This cross-sectional study provides a detailed characterization of the International Cooperative Ataxia Rating Scale. The analysis further provides rates of change separated for patients with early and late disease onset, which is driven by the GAA repeat length. Differences in the subscale dynamics merit consideration in the design of future clinical trials applying this scale as a neurological assessment instrument in Friedreich's ataxia.
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