ArticleAnnals of clinical and translational neurology2020
Age of onset determines intrinsic functional brain architecture in Friedreich ataxia.
Article in Annals of clinical and translational neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 27 citations in OpenAlex.
- Listening in Spatialized Noise-Sentences (LiSN-S) as a Measure of Auditory Function in Friedreich Ataxia.Cerebellum (London, England) · 2026Article
- The Dynamic Interaction between Procedural Learning and Resting-State Functional Connectivity in Self-Limited Focal Epilepsies.Brain topography · 2025Article
- Altered Intracerebellar Functional Connectivity in Friedreich's Ataxia: A Graph-Theory Functional MRI Study.Cerebellum (London, England) · 2025Article
- Lean body mass and stroke volume, a sex issue.Frontiers in neurology · 2024Article
- The dissociative role of bursting and non-bursting neural activity in the oscillatory nature of functional brain networks.Imaging neuroscience (Cambridge, Mass.) · 2024Article
- Physiological Recordings of the Cerebellum in Movement Disorders.Cerebellum (London, England) · 2023Review
- Retinal hypoplasia and degeneration result in vision loss in Friedreich ataxia.Annals of clinical and translational neurology · 2023Article
- Determinant of the cerebellar cognitive affective syndrome in Friedreich's ataxia.Journal of neurology · 2023Observational
- Reduced cerebello-cerebral functional connectivity correlates with disease severity and impaired white matter integrity in Friedreich ataxia.Journal of neurology · 2023Article
- Neurophysiology of cerebellar ataxias and gait disorders.Clinical neurophysiology practice · 2023Review
- Friedreich ataxia: clinical features and new developments.Neurodegenerative disease management · 2022Review
- Microstates and power envelope hidden Markov modeling probe bursting brain activity at different timescales.NeuroImage · 2022Article
- Cerebrospinal Fluid Proteomics in Friedreich Ataxia Reveals Markers of Neurodegeneration and Neuroinflammation.Frontiers in neuroscience · 2022Article
- Atypical resting-state functional brain connectivity in children with developmental coordination disorder.NeuroImage. Clinical · 2022Article
- Cerebellar Pathology in an Inducible Mouse Model of Friedreich Ataxia.Frontiers in neuroscience · 2022Article
- Cerebellar cognitive affective syndrome after acute cerebellar stroke.Frontiers in neurology · 2022Article
- Age of onset modulates resting-state brain network dynamics in Friedreich Ataxia.Human brain mapping · 2021Article
- Huntington's disease: lessons from prion disorders.Journal of neurology · 2021Article
- Brain dysconnectivity relates to disability and cognitive impairment in multiple sclerosis.Human brain mapping · 2021Article
- Changes in electrophysiological static and dynamic human brain functional architecture from childhood to late adulthood.Scientific reports · 2020Article
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
objectiveFriedreich ataxia (FRDA) is the commonest hereditary ataxia in Caucasians. Most patients are homozygous for expanded GAA triplet repeats in the first intron of the frataxin (FXN) gene, involved in mitochondrial iron metabolism. Here, we used magnetoencephalography (MEG) to characterize the main determinants of FRDA-related changes in intrinsic functional brain architecture.
methodsFive minutes of MEG signals were recorded at rest from 18 right-handed FRDA patients (mean age 27 years, 9 females; mean SARA score: 21.4) and matched healthy individuals. The MEG connectome was estimated as resting-state functional connectivity (rsFC) matrices involving 37 nodes from six major resting state networks and the cerebellum. Source-level rsFC maps were computed using leakage-corrected broad-band (3-40 Hz) envelope correlations. Post hoc median-split was used to contrast rsFC in FRDA patients with different clinical characteristics. Nonparametric permutations and Spearman rank correlation test were used for statistics.
resultsHigh rank correlation levels were found between rsFC and age of symptoms onset in FRDA mostly between the ventral attention, the default-mode, and the cerebellar networks; patients with higher rsFC developing symptoms at an older age. Increased rsFC was found in FRDA with later age of symptoms onset compared to healthy subjects. No correlations were found between rsFC and other clinical parameters.
conclusionAge of symptoms onset is a major determinant of FRDA patients' intrinsic functional brain architecture. Higher rsFC in FRDA patients with later age of symptoms onset supports compensatory mechanisms for FRDA-related neural network dysfunction and position neuromagnetic rsFC as potential marker of FRDA neural reserve.
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