Observational studyAnnals of clinical and translational neurology2021
Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort.
Observational study in Annals of clinical and translational neurology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
18 citing papers in PubMed, 34 citations in OpenAlex.
- Neuropathology of Friedreich ataxia and its links to metabolic pathways.Neurodegenerative disease management · 2026Review
- Serial assessment of echocardiographic measures in Friedreich ataxia.American heart journal plus : cardiology research and practice · 2026Article
- Disease Progression in Children With Friedreich Ataxia: Functional Performance and Other Outcome Assessments in the FACHILD Study.Journal of child neurology · 2026Article
- Coexistence of Friedreich's Ataxia and Esophageal Cancer: A Case Report.Clinical case reports · 2026Article
- Scoliosis Surgery in a Patient With Advanced Friedreich's Ataxia-It Is Not Too Late.Annals of clinical and translational neurology · 2026Article
- Genome-wide association study identifiesPeerJ · 2026Article
- Perioperative Anesthetic Management in Pediatric Scoliosis Surgery: A Narrative Review with Focus on Neuromuscular Disorders.Children (Basel, Switzerland) · 2025Review
- Friedreich ataxia: what can we learn from non-GAA repeat mutations?Neurodegenerative disease management · 2025Review
- Frataxin deficiency and the pathology of Friedreich's Ataxia across tissues.Tissue barriers · 2025Review
- Longitudinal analysis of anthropometric measures over 5 years in patients with Friedreich ataxia in the EFACTS natural history study.European journal of neurology · 2025Article
- Pulling back the mitochondria's iron curtain.npj metabolic health and disease · 2025Review
- Case Report: Complex cardiac arrhythmia management in the ICU for an adolescent with Friedreich ataxia.Frontiers in pediatrics · 2025Article
- Identification of an increased lifetime risk of major adverse cardiovascular events in UK Biobank participants with scoliosis.Open heart · 2023Article
- Cardiovascular Research in Friedreich Ataxia: Unmet Needs and Opportunities.JACC. Basic to translational science · 2022Review
- Friedreich ataxia: clinical features and new developments.Neurodegenerative disease management · 2022Review
- Respiratory Function in Friedreich's Ataxia.Children (Basel, Switzerland) · 2022Article
- Clinical Evidence for Variegated Silencing in Patients With Friedreich Ataxia.Neurology. Genetics · 2022Article
- Friedreich's Ataxia related Diabetes: Epidemiology and management practices.Diabetes research and clinical practice · 2022Article
Corrections and comments
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Authors and funding
17 authors at 15 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveThe objective of this study was to characterize the incidence and progression of scoliosis in the natural history of Friedreich's ataxia (FRDA) and document the factors leading to the requirement for corrective surgery.
methodsData on the prevalence of scoliosis and scoliosis surgery from up to 17 years of follow-up collected during a large natural history study in FRDA (1116 patients at 4928 visits) were summarized descriptively and subjected to time to event analyses.
resultsWell over 90% of early or typical FRDA patients (as determined by age of onset) developed intermediate to severe scoliosis, while patients with a later onset (>14 years) had no or much lower prevalence of scoliosis. Diagnosis of scoliosis occurs during the onset of ataxia and in rare cases even prior to that. Major progression follows throughout the growth phase and puberty, leading to the need for surgical intervention in more than 50% of individuals in the most severe subgroup. The youngest patients appear to delay surgery until the end of the growth period, leading to further progression before surgical intervention. Age of onset of FRDA before or after reaching 15 years sharply separated severe and relatively mild incidence and progression of scoliosis.
interpretationScoliosis is an important comorbidity of FRDA. Our comprehensive documentation of scoliosis progression in this natural history study provides a baseline for comparison as novel treatments become available.
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