The Cerebellum

, Volume 17, Issue 3, pp 336–345 | Cite as

Emotion Recognition and Psychological Comorbidity in Friedreich’s Ataxia

  • Teresa Costabile
  • Veronica Capretti
  • Filomena Abate
  • Agnese Liguori
  • Francesca Paciello
  • Chiara Pane
  • Anna De Rosa
  • Silvio Peluso
  • Giuseppe De Michele
  • Alessandro Filla
  • Francesco Saccà
Original Paper


Friedreich’s ataxia (FRDA) is an autosomal recessive disease presenting with ataxia, corticospinal signs, peripheral neuropathy, and cardiac abnormalities. Little effort has been made to understand the psychological and emotional burden of the disease. The aim of our study was to measure patients’ ability to recognize emotions using visual and non-verbal auditory hints, and to correlate this ability with psychological, neuropsychological, and neurological variables. We included 20 patients with FRDA, and 20 age, sex, and education matched healthy controls (HC). We measured emotion recognition using the Geneva Emotion Recognition Test (GERT). Neuropsychological status was assessed measuring memory, executive functions, and prosopagnosia. Psychological tests were Patient Health Questionnaire-9 (PHQ-9), State Trait Anxiety Inventory–state/−trait (STAI-S/−T), and Structured Clinical Interview for DSM Disorders II. FRDA patients scored worse at the global assessment and showed impaired immediate visuospatial memory and executive functions. Patients presented lower STAI-S scores, and similar scores at the STAI-T, and PHQ-9 as compared to HC. Three patients were identified with personality disorders. Emotion recognition was impaired in FRDA with 29% reduction at the total GERT score (95% CI − 44.8%, − 12.6%; p < 0.001; Cohen’s d = 1.2). Variables associated with poor GERT scores were the 10/36 spatial recall test, the Ray Auditory Verbal Learning Test, the Montreal Cognitive Assessment, and the STAI-T (R2 = 0.906; p < 0.001). FRDA patients have impaired emotion recognition that may be secondary to neuropsychological impairment. Depression and anxiety were not higher in FRDA as compared to HC and should not be considered as part of the disease.




Authors’ Contribution

A. Design and conceptualization of the study.

B. Analysis or interpretation of the data.

C. Drafting or revising the manuscript for intellectual content.

Teresa Costabile ABC, Veronica Capretti B, Filomena Abate B, Agnese Liguori B, Francesca Paciello B, Chiara Pane AB, Anna De Rosa AB, Silvio Peluso C, Giuseppe De Michele C, Alessandro Filla C, Francesco Saccà ABC.

Funding Information

This study did not receive any financial support.

Compliance with Ethical Standards

Authors’ Disclosures

All authors have nothing to disclose.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Teresa Costabile
    • 1
  • Veronica Capretti
    • 1
  • Filomena Abate
    • 1
  • Agnese Liguori
    • 1
  • Francesca Paciello
    • 1
  • Chiara Pane
    • 1
  • Anna De Rosa
    • 1
  • Silvio Peluso
    • 1
  • Giuseppe De Michele
    • 1
  • Alessandro Filla
    • 1
  • Francesco Saccà
    • 1
  1. 1.Department of Neurosciences and Reproductive and Odontostomatological SciencesUniversity “Federico II”NaplesItaly

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