Health-related quality of life and depressive symptoms in Friedreich ataxia



Friedreich ataxia (FRDA) is a chronic, progressive and highly disabling cerebellar degenerative disease. Despite this, little attention has been paid to the health-related quality of life (HRQOL) in this disease. The aim of the present study was to assess FRDA patients’ perception of HRQOL and to determine the influence of depression, and demographic and clinical variables.


The sample consisted of 62 patients with genetically confirmed FRDA. The SF-36 Health Survey was used to assess HRQOL. Depressive symptoms were evaluated with the Beck Depression Inventory-II.


FRDA patients’ mean scores were significantly lower than the values for the Spanish population in all SF36 dimensions. Average z scores ranged from − 5.5 in physical functioning to − 0.48 in mental health. Age and clinical variables were significant predictors of HRQOL in only several dimensions, whereas BDI scores were able to predict a significant percentage of variance in all SF36 dimensions, except physical functioning.


Our study demonstrates the high impact of Friedreich ataxia on quality of life. This impact does not only occur in those aspects most related to motor disability but it is also present in non-motor dimensions. Depressive symptomatology is the most relevant variable for predicting quality of life.

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The authors thank Dr. Berciano (Hospital Marqués de Valdecillas, Santander) and Dr. Arpa (Hospital La Paz, Madrid) for providing access to patents and for their helpful assistance.


This work was supported by the Ministerio de Economía y Competitividad (Spain) [Grant Number PSI 2015-67514-P].

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Correspondence to Antonieta Nieto.

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Pérez-Flores, J., Hernández-Torres, A., Montón, F. et al. Health-related quality of life and depressive symptoms in Friedreich ataxia. Qual Life Res 29, 413–420 (2020).

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  • Friedreich ataxia
  • Cerebellum
  • Quality of life
  • Depression
  • Neurological diseases