ArticleCerebellum (London, England)2011
Superior cerebellar peduncle atrophy in Friedreich's ataxia correlates with disease symptoms.
Article in Cerebellum (London, England), 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers.
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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.
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Who cites it
38 citing papers in PubMed, 70 citations in OpenAlex.
- White matter changes in patients with friedreich ataxia after treatment with erythropoietin.Journal of neuroimaging : official journal of the American Society of NeuroimagingTrial
- MRI end-points for clinical trials in ataxias: recommendations from the Ataxia Global Initiative MRI Biomarkers Working Group.Nature reviews. Neurology · 2026Review
- An overview of early-onset cerebellar ataxia: a practical guideline.Acta neurologica Belgica · 2024Review
- New and Emerging Drug and Gene Therapies for Friedreich Ataxia.CNS drugs · 2024Review
- Reduced cerebello-cerebral functional connectivity correlates with disease severity and impaired white matter integrity in Friedreich ataxia.Journal of neurology · 2023Article
- Brain MRI detects early-stage alterations and disease progression in Friedreich ataxia.Brain communications · 2023Article
- Increased brain tissue sodium concentration in Friedreich ataxia: A multimodal MR imaging study.NeuroImage. Clinical · 2022Article
- A natural history study to track brain and spinal cord changes in individuals with Friedreich's ataxia: TRACK-FA study protocol.PloS one · 2022Article
- Longitudinal structural brain changes in Friedreich ataxia depend on disease severity: the IMAGE-FRDA study.Journal of neurology · 2021Article
- Neuro-Ophthalmological Findings in Friedreich's Ataxia.Journal of personalized medicine · 2021Review
- Central Nervous System Therapeutic Targets in Friedreich Ataxia.Human gene therapy · 2020Review
- Toward quantitative neuroimaging biomarkers for Friedreich's ataxia at 7 Tesla: Susceptibility mapping, diffusion imaging, RJournal of neuroscience research · 2020Article
- Test-retest reliability of the Friedreich's ataxia rating scale.Annals of clinical and translational neurology · 2020Article
- Multiple mechanisms underpin cerebral and cerebellar white matter deficits in Friedreich ataxia: The IMAGE-FRDA study.Human brain mapping · 2020Article
- Sensitivity of Neuroimaging Indicators in Monitoring the Effects of Interferon Gamma Treatment in Friedreich's Ataxia.Frontiers in neuroscience · 2020Article
- Atrophy of cerebellar peduncles in essential tremor: a machine learning-based volumetric analysis.European radiology · 2019Article
- The Cerebello-Hypothalamic and Hypothalamo-Cerebellar Pathways via Superior and Middle Cerebellar Peduncle in the Rat.Cerebellum (London, England) · 2018Article
- Cognitive and functional connectivity alterations in Friedreich's ataxia.Annals of clinical and translational neurology · 2018Article
- Functional and Structural Brain Damage in Friedreich's Ataxia.Frontiers in neurology · 2018Article
- Tremor in multiple sclerosis is associated with cerebello-thalamic pathology.Journal of neural transmission (Vienna, Austria : 1996) · 2017Article
Corrections and comments
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Authors and funding
7 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Friedreich's ataxia (FRDA) is the most common early onset inherited ataxia with clinical manifestations, including gradual progression of unremitting cerebellar-sensory ataxia, peripheral sensory loss, loss of lower limb tendon reflexes and hypertrophic cardiomyopathy. Although atrophy of the superior cerebellar peduncle (SCP) has been reported in several magnetic resonance imaging (MRI) studies of FRDA, the relationship of SCP changes to genetic and clinical features of FRDA has not been investigated. We acquired T1-weighted MRI scans in 12 right-handed individuals with FRDA, homozygous for a GAA expansion in intron 1 of FXN, as well as 13 healthy age-matched controls. The corrected cross-sectional areas of the right (left) SCP in the individuals with FRDA (R, 20 ± 7.9 mm(2); L, 25 ± 5.6 mm(2)) were significantly smaller than for controls (R, 68 ± 16 mm(2); L, 78 ± 17 mm(2)) (p < 0.001). The SCP volumes of individuals with FRDA were negatively correlated with Friedreich's ataxia rating scale score (r = -0.553) and disease duration (r = -0.541), and positively correlated with the age of onset (r = 0.548) (p < 0.05). These findings suggest that structural MR imaging of the SCP can provide a surrogate marker of disease severity in FRDA and support the potential role of structural MRI as a biomarker in the evaluation of neurodegenerative diseases and therapies.
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