ArticleCerebellum (London, England)2018
Personality and Neuropsychological Profiles in Friedreich Ataxia.
Article in Cerebellum (London, England), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 2 of them syntheses that pooled it.
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
19 citing papers in PubMed, 2 syntheses or guidelines pooled it, 30 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
- Cerebellar cognitive-affective syndrome in Friedreich Ataxia.Journal of neurology · 2026Observational
- A Patient-Reported Outcome Measure of Communication Difficulties in Friedreich Ataxia: COMATAX.Cerebellum (London, England) · 2026Article
- Patient-reported, psychosocial and health economic outcomes in mild to moderate Friedreich's ataxia: baseline results of the PROFA study.The Lancet regional health. Europe · 2026Article
- Spatial perspective taking is impaired in spinocerebellar ataxias and Friedreich ataxia.Scientific reports · 2025Article
- Cerebellar dysfunction in frontotemporal dementia: intra-cerebellar pathology and cerebellar network degeneration.Journal of neurology · 2025Article
- Clinical and cognitive assessment in Friedreich ataxia clinical trials: a review.Frontiers in neurology · 2025Review
- Exploring neuropsychiatric symptoms in Friedreich ataxia.Scientific reports · 2024Article
- Determinant of the cerebellar cognitive affective syndrome in Friedreich's ataxia.Journal of neurology · 2023Observational
- Friedreich ataxia: clinical features and new developments.Neurodegenerative disease management · 2022Review
- The CCAS-scale in hereditary ataxias: helpful on the group level, particularly in SCA3, but limited in individual patients.Journal of neurology · 2022Article
- Cognitive Dysfunction in Repeat Expansion Diseases: A Review.Frontiers in aging neuroscience · 2022Review
- Friedreich Ataxia: Multidisciplinary Clinical Care.Journal of multidisciplinary healthcare · 2021Review
- Clinical, neuropathological, and genetic characterization of STUB1 variants in cerebellar ataxias: a frequent cause of predominant cognitive impairment.Genetics in medicine : official journal of the American College of Medical Genetics · 2020Article
- Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia.Annals of clinical and translational neurology · 2020Article
- Longitudinal Increases in Cerebral Brain Activation During Working Memory Performance in Friedreich Ataxia: 24-Month Data from IMAGE-FRDA.Cerebellum (London, England) · 2020Article
- Speech and Language Disorders in Friedreich Ataxia: Highlights on Phenomenology, Assessment, and Therapy.Cerebellum (London, England) · 2020Review
- Commentary: Novelty seeking and reward dependence-related large-scale brain networks functional connectivity variation during salience expectancy.Frontiers in psychology · 2018Article
Corrections and comments
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Authors and funding
12 authors at 7 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Friedreich ataxia, an autosomal recessive mitochondrial disease, is the most frequent inherited ataxia. Many studies have attempted to identify cognitive and affective changes associated with the disease, but conflicting results have been obtained, depending on the tests used and because many of the samples studied were very small. We investigated personality and neuropsychological characteristics in a cohort of 47 patients with genetically confirmed disease. The neuropsychological battery assessed multiple cognition domains: processing speed, attention, working memory, executive functions, verbal memory, vocabulary, visual reasoning, emotional recognition, and social cognition. Personality was assessed with the Temperament and Character Inventory, and depressive symptoms were assessed with the Beck Depression Inventory. We found deficits of sustained attention, processing speed, semantic capacities, and verbal fluency only partly attributable to motor deficit or depressed mood. Visual reasoning, memory, and learning were preserved. Emotional processes and social cognition were unimpaired. We also detected a change in automatic processes, such as reading. Personality traits were characterized by high persistence and low self-transcendence. The mild cognitive impairment observed may be a developmental rather than degenerative problem, due to early cerebellum dysfunction, with the impairment of cognitive and emotional processing. Disease manifestations at crucial times for personality development may also have an important impact on personality traits.
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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.