RT info:eu-repo/semantics/article
T1 Longitudinal study of cognitive functioning in Friedreich's Ataxia
A1 Hernández Torres, Atteneri
A1 Montón, Fernando
A1 Hess Medler, Stephany
A1 Nóbrega, Érika de
A1 Nieto Barco, Antonieta
A2 Psicología ClínicaPsicobiología y Metodología
A2 Grupo de investigación ULL: Neuropsicología
Facultad de Psicología y Logopedia
Instituto Universitario de Neurociencia
K1 Friedreich’s ataxia
K1 Longitudinal study
K1 Cognition
K1 Neuropsychology
K1 Cerebellum
AB Objective: Friedreich’s ataxia (FRDA) is the most common hereditary ataxia. It is a neurodegenerative disorder,characterized by progressive ataxia. FRDA is also associated with cognitive impairments. To date, the evolution ofcognitive functioning is unknown. Our aim was to investigate the changes in the cognitive functioning of FRDApatients over an average eight-year timeframe. In addition, we aimed to study the relationship between cognitivechanges and clinical variables. Methods: Twenty-nine FRDA patients who had been part of the sample of a previousstudy participated in the present study. The mean average time between the two assessments was 8.24 years. Theparticipants completed an extensive battery of neuropsychological tests chosen to examine cognitive functioning invarious cognitive domains: processing speed, attention, working memory, executive functions, verbal and visualmemory, visuoperceptive and visuospatial skills, visuoconstructive functions and language. Results: At follow-up,cerebellar symptoms had worsened, and patients presented greater disability. Differences between baseline and followup were observed in motor and cognitive reaction times, several trials of the Stroop test, semantic fluency, and blockdesigns. No other cognitive changes were observed. Deterioration in simple cognitive reactions times and block designsperformance correlated with the progression of cerebellar symptoms. Conclusions: Our study has demonstrated for thefirst time that patients with FRDA experience a significant decline over time in several cognitive domains. Specifically,after an eight-year period, FRDA patients worsened in processing speed, fluency, and visuoconstructive skills. Thisprogression is unlikely to be due to greater motor or speech impairment.
YR 2021
FD 2021
LK http://riull.ull.es/xmlui/handle/915/35389
UL http://riull.ull.es/xmlui/handle/915/35389
LA en
NO doi:10.1017/S1355617720000958
DS Repositorio institucional de la Universidad de La Laguna
RD 18-may-2024