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Longitudinal Study of Cognitive Functioning in Friedreich’s Ataxia

Published online by Cambridge University Press:  14 October 2020

Atteneri Hernández-Torres ,
Fernando Montón ,
Stephany Hess Medler ,
Érika de Nóbrega  and
Antonieta Nieto Open the ORCID record for Antonieta Nieto [Opens in a new window]
Atteneri Hernández-Torres
Affiliation:
Facultad de Psicología, Universidad de La Laguna (ULL), Campus de Guajara, 38200 La Laguna (Tenerife), España
Fernando Montón
Affiliation:
Departamento de Neurología, Hospital Nuestra Señora de Candelaria (HUNSC), 38010 Santa Cruz de Tenerife, España
Stephany Hess Medler
Affiliation:
Facultad de Psicología, Universidad de La Laguna (ULL), Campus de Guajara, 38200 La Laguna (Tenerife), España
Érika de Nóbrega
Affiliation:
Facultad de Psicología, Universidad de La Laguna (ULL), Campus de Guajara, 38200 La Laguna (Tenerife), España
Antonieta Nieto*
Affiliation:
Facultad de Psicología, Universidad de La Laguna (ULL), Campus de Guajara, 38200 La Laguna (Tenerife), España
*
*Correspondence and reprint requests to: Antonieta Nieto, School of Psychology, University of La Laguna, 38205La Laguna, Tenerife, Spain. E-mail: [email protected]
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Abstract

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 of cognitive functioning is unknown. Our aim was to investigate the changes in the cognitive functioning of FRDA patients over an average eight-year timeframe. In addition, we aimed to study the relationship between cognitive changes and clinical variables.

Methods:

Twenty-nine FRDA patients who had been part of the sample of a previous study participated in the present study. The mean average time between the two assessments was 8.24 years. The participants completed an extensive battery of neuropsychological tests chosen to examine cognitive functioning in various cognitive domains: processing speed, attention, working memory, executive functions, verbal and visual memory, 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 follow-up were observed in motor and cognitive reaction times, several trials of the Stroop test, semantic fluency, and block designs. No other cognitive changes were observed. Deterioration in simple cognitive reactions times and block designs performance correlated with the progression of cerebellar symptoms.

Conclusions:

Our study has demonstrated for the first 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. This progression is unlikely to be due to greater motor or speech impairment.

Keywords

Friedreich’s ataxiaLongitudinal studyCognitionNeuropsychologyCerebellum
Type
Regular Research
Information
Journal of the International Neuropsychological Society , Volume 27 , Issue 4 , April 2021 , pp. 343 - 350
Copyright
Copyright © INS. Published by Cambridge University Press, 2020

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