Neuropsychological profile of hereditary ataxias: study of 38 patients

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dc.contributor.authorGarcía Martín, Maitane
dc.contributor.authorRouco Axpe, Idoia
dc.contributor.authorAmayra Caro, Imanol
dc.contributor.authorRodríguez-Antigüedad Zarranz, Alfredo
dc.contributor.authorCatalli, Claudio
dc.contributor.authorCabrera-Zubizarreta, Alberto
dc.contributor.authorRodríguez Bermejo, Alicia Aurora
dc.contributor.authorPérez Álvarez, Manuel
dc.date.accessioned2024-11-08T12:16:33Z
dc.date.available2024-11-08T12:16:33Z
dc.date.issued2022-08
dc.date.updated2024-11-08T12:16:33Z
dc.description.abstractHereditary ataxias are a heterogeneous group of disorders characterized by degeneration of the cerebellum and its connections. It is known that patients with ataxia can manifest a broad spectrum of motor symptoms; however, current research has emphasized the relevance of cognitive disturbances. The aim of this study is to analyze the presence of cognitive impairment in a heterogeneous cohort of patients with hereditary ataxia (HA). A group of 38 patients with HA of different etiologies and a matched group of 38 healthy controls were recruited and evaluated through a comprehensive battery of neuropsychological tests. The findings show a worse performance in ataxic patients on planning, visuospatial skills, naming, and Theory of Mind tasks, regardless their physical and psychological symptomatology. The influence of clinical status as well as functional ability-related variables on their performance were analyzed, showing that the level of disability and motor disturbances have a significant effect on verbal memory, verbal fluency, and working memory. These findings suggest that patients with HA can manifest cognitive and neuropsychiatric symptoms as part of their clinical features, which demands its inclusion for the diagnosis and management of the disease.en
dc.identifier.citationGarcía, M., Rouco-Axpe, I., Amayra, I., Rodríguez-Antigüedad, A., Catalli, C., Cabrera-Zubizarreta, A., Rodríguez, A. A., & Pérez, M. (2022). Neuropsychological Profile of Hereditary Ataxias: Study of 38 Patients. Archives of Clinical Neuropsychology, 37(5), 904-915. https://doi.org/10.1093/ARCLIN/ACAC024
dc.identifier.doi10.1093/ARCLIN/ACAC024
dc.identifier.issn1873-5843
dc.identifier.urihttp://hdl.handle.net/20.500.14454/1715
dc.language.isoeng
dc.publisherOxford University Press
dc.rights© The Author(s) 2022
dc.subject.otherCerebellum
dc.subject.otherCognition
dc.subject.otherHereditary ataxia
dc.subject.otherSocial cognition
dc.titleNeuropsychological profile of hereditary ataxias: study of 38 patientsen
dc.typejournal article
dcterms.accessRightsmetadata only access
oaire.citation.endPage915
oaire.citation.issue5
oaire.citation.startPage904
oaire.citation.titleArchives of Clinical Neuropsychology
oaire.citation.volume37
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