ArticleAnnals of clinical and translational neurology2016
Cognition in Friedreich's ataxia: a behavioral and multimodal imaging study.
Article in Annals of clinical and translational neurology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 4 of them syntheses that pooled it.
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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
38 citing papers in PubMed, 4 syntheses or guidelines pooled it, 61 citations in OpenAlex.
- Cognition in cerebellar disorders: What's in the profile? A systematic review and meta-analysis.Journal of neurology · 2025Pooled it
- The cognitive profile of Friedreich ataxia: a systematic review and meta-analysis.BMC neurology · 2022Pooled it
- Functional MRI Studies in Friedreich's Ataxia: A Systematic Review.Frontiers in neurology · 2021Pooled it
- The Cerebellum in Frontotemporal Dementia: a Meta-Analysis of Neuroimaging Studies.Neuropsychology review · 2019Pooled it
- Protocol of a randomized, double-blind, placebo-controlled, parallel-group, multicentre study of the efficacy and safety of nicotinamide in patients with Friedreich ataxia (NICOFA).Neurological research and practice · 2019Trial
- Multimodal Imaging Investigation of the Dentato-Thalamo-Cortical Pathway in Friedreich's Ataxia.Movement disorders : official journal of the Movement Disorder Society · 2026Article
- Hereditary Ataxias: From Pathogenesis and Clinical Features to Neuroimaging, Fluid, and Digital Biomarkers-A Scoping Review.International journal of molecular sciences · 2026Review
- False Beliefs, True Deficits: Investigating Social Cognition in Friedreich Ataxia.Cerebellum (London, England) · 2025Article
- Optimizing selectivity of the Cerebellar Cognitive Affective Syndrome Scale by use of correction formulas, and validation of its German version.Journal of neurology · 2025Article
- Muscle Endurance Training in a Person with Friedreich's Ataxia.Muscles (Basel, Switzerland) · 2025Article
- Subtypes of cognitive impairment in cerebellar disease identified by cross-diagnostic cluster-analysis: results from a German multicenter study.Journal of neurology · 2024Article
- Exploring neuropsychiatric symptoms in Friedreich ataxia.Scientific reports · 2024Article
- Cognitive impairment associated with cerebellar volume loss in spinocerebellar ataxia type 3.Journal of neurology · 2024Article
- The significance of cerebellar contributions in early-life through aging.Frontiers in computational neuroscience · 2024Article
- Reduced cerebello-cerebral functional connectivity correlates with disease severity and impaired white matter integrity in Friedreich ataxia.Journal of neurology · 2023Article
- Social concepts and the cerebellum: behavioural and functional connectivity signatures in cerebellar ataxic patients.Philosophical transactions of the Royal Society of London. Series B, Biological sciences · 2023Article
- Brain MRI detects early-stage alterations and disease progression in Friedreich ataxia.Brain communications · 2023Article
- The CCAS-scale in hereditary ataxias: helpful on the group level, particularly in SCA3, but limited in individual patients.Journal of neurology · 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
- Increased brain tissue sodium concentration in Friedreich ataxia: A multimodal MR imaging study.NeuroImage. Clinical · 2022Article
Corrections and comments
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Authors and funding
14 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveFriedreich's ataxia (FRDA) is a spinocerebellar degenerative disorder, in which cognitive deficits are sparsely explored. In this behavioral and multimodal magnetic resonance imaging (MRI) study, we investigated the neurocognitive profile and cortico-cerebellar dysfunctions underlying executive functioning in individuals with FRDA.
methods22 FRDA patients and 22 controls were clinically and neuropsychologically examined. Fifteen of each underwent structural and functional MRI using a verbal-fluency task with phonemic and semantic conditions. Gray (GM) and white matter (WM) alterations were assessed by means of voxel-based morphometry and diffusion-tensor imaging.
resultsThe neuropsychological profile demonstrated deficits in verbal fluency, working memory and social cognition. Functional MRI data showed most pronounced group-differences in phonemic fluency with patients exhibiting enhanced activity in the cerebellum (VI, Crus I), fronto-insular, premotor and temporo-occipital regions. The semantic condition only revealed reduced activity in the anterior cerebellum; for overt speech, we found increased activity in the motor cortex. Functional connectivity-analysis showed higher co-activation within cerebellar and cortical regions, respectively, and impaired interregional coupling between the cerebellum and fronto-insular cortex for phonemic processing, which was also related to poorer task performance. GM reduction in FRDA was mainly found in lobule VI, whereas WM degeneration was more pronounced including brainstem, cerebellum, and cortex. Decreased cerebellar GM was associated with enhanced activity in the fronto-insular cortex, while loss of WM integrity may translate cortico-cerebellar pathway disruptions.
interpretationThe pattern of increased neural response with both cerebellar and cortical involvement underlying executive functioning indicates functional reorganization driven by disease-related structural damage in FRDA.
Identifiers
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.