Clinical Management Guidelines for Friedreich Ataxia (FRDA)

Chapter 14. Mental health issues in Friedreich ataxia

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This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.

14.1 Literature review of prevalence of mental health issues in Friedreich ataxia

14.2 Management of depression in Friedreich ataxia

14.3 Management of anxiety in Friedreich ataxia

14.4 Management of psychosis in Friedreich ataxia

Disclaimer / Intended Use / Funding

Disclaimer
The Clinical Management Guidelines for Friedreich ataxia (‘Guidelines’) are protected by copyright owned by the authors who contributed to their development or said authors’ assignees.

These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.

Guidelines users must seek out the most recent information that might supersede the diagnostic and treatment recommendations contained within these Guidelines and consider local variations in clinical settings, funding and resources that may impact on the implementation of the recommendations set out in these Guidelines.

The authors of these Guidelines disclaim all liability for the accuracy or completeness of the Guidelines, and disclaim all warranties, express or implied to their incorrect use.

Intended Use
These Guidelines are made available as general information only and do not constitute medical advice. These Guidelines are intended to assist qualified healthcare professionals make informed treatment decisions about the care of individuals with Friedreich ataxia. They are not intended as a sole source of guidance in managing issues related to Friedreich ataxia. Rather, they are designed to assist clinicians by providing an evidence-based framework for decision-making.

These Guidelines are not intended to replace clinical judgment and other approaches to diagnosing and managing problems associated with Friedreich ataxia which may be appropriate in specific circumstances. Ultimately, healthcare professionals must make their own treatment decisions on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise.
Guidelines users must not edit or modify the Guidelines in any way – including removing any branding, acknowledgement, authorship or copyright notice.

Funding
The authors of this document gratefully acknowledge the support of the Friedreich Ataxia Research Alliance (FARA). The views and opinions expressed in the Guidelines are solely those of the authors and do not necessarily reflect the official policy or position of FARA.


14. Mental health issues in Friedreich ataxia

This chapter describes important mental health issues for individuals with Friedreich ataxia, including depression, anxiety and psychosis, and strategies for investigation and management. In making recommendations for management of these mental health conditions, the authors were tasked with answering the following questions:

For individuals with Friedreich ataxia, what is the best management for depression? (see 14.2)

For individuals with Friedreich ataxia, what is the best management for anxiety? (see 14.3)

For individuals with Friedreich ataxia, what is the best management for psychosis? (see 14.4)

Jennifer Farmer is acknowledged for much of the content of the literature review and overview of depression, taken from the previous version of the guidelines (2014).

Louise Corben, PhD
Principal Research Fellow, Murdoch Children’s Research Institute, Melbourne, Victoria, Australia
Email: louise.corben@mcri.edu.au

Alexandra Durr, MD, PhD
Professor of Neurogenetics, Sorbonne Université, Paris, France
Email: alexandra.durr@icm-institute.org

Susan Perlman, MD
Clinical Professor of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
Email: sperlman@ucla.edu

Caroline Spencer, PhD
Postdoctoral Fellow, Boston University, Boston, Massachusetts, USA
Email: cspencer@bu.edu

George Wilmot, MD, PhD
Associate Professor, Department of Neurology, Emory University, Atlanta, Georgia, USA
Email: gwilmot@emory.edu

1. Flood MK, Perlman SL. The mental status of patients with Friedreich’s ataxia. J Neurosci Nurs. 1987;19(5):251-5.

2. Giordani B, Boivan M, Berent S, Gilman S, Junck L, Lehtinen S, et al. Cognitive and emotional function in Friedreich’s ataxia (Abstract). J Clin Exp Neuropsychol. 1989;11(1):53-4.

3. Mantovan MC, Martinuzzi A, Squarzanti F, Bolla A, Silvestri I, Liessi G, et al. Exploring mental status in Friedreich’s ataxia: a combined neuropsychological, behavioural and neuroimaging study. Eur J Neurol. 2006;13:827-35.

4. White M, Lalonde R, Botez-Marquard T. Neuropsychologic and neuropsychiatric characteristics of patients with Friedreich’s ataxia. Acta Neurol Scand. 2000;102(4):222-6.

5. Ciancarelli I, Cofini V, Carolei A. Evaluation of neuropsychological functions in patients with Friedreich ataxia before and after cognitive therapy. Funct Neurol. 2010;25(2):81-5.

6. Sayah S, Rotge JY, Francisque H, Gargiulo M, Czernecki V, Justo D, et al. Personality and neuropsychological profiles in Friedreich ataxia. Cerebellum. 2018;17(2):204-12.

7. Corben LA, Delatycki MB, Bradshaw JL, Horne MK, Fahey MC, Churchyard AC, et al. Impairment in motor reprogramming in Friedreich ataxia reflecting possible cerebellar dysfunction. J Neurol. 2010;257(5):782-91.

8. Corben LA, Delatycki MB, Bradshaw JL, Churchyard AJ, Georgiou-Karistianis N. Utilisation of advance motor information is impaired in Friedreich ataxia. Cerebellum. 2011;10(4):793-803.

9. Corben LA, Akhlaghi H, Georgiou-Karistianis N, Bradshaw JL, Egan GF, Storey E, et al. Impaired inhibition of prepotent motor tendencies in Friedreich ataxia demonstrated by the Simon interference task. Brain Cogn. 2011;76(1):140-5.

10. Corben LA, Georgiou-Karistianis N, Bradshaw JL, Hocking DR, Churchyard AJ, Delatycki MB. The Fitts task reveals impairments in planning and online control of movement in Friedreich ataxia: reduced cerebellar-cortico connectivity? Neuroscience 2011;192(382-390).

11. White BV, Leib JR, Farmer JM, Biesecker BB. Exploration of transitional life events in individuals with Friedreich ataxia: implications for genetic counseling. Behav Brain Funct. 2010;6(65).

12. da Silva CB, Yasuda CL, D’Abreu A, Cendes F, Lopes-Cendes I, Franca MC, Jr. Neuroanatomical correlates of depression in Friedreich’s ataxia: a voxel-based morphometry study. Cerebellum. 2013;12(3):429-36.

13. Nieto A, Hernandez-Torres A, Perez-Flores J, Monton F. Depressive symptoms in Friedreich ataxia. Int J Clin Health Psychol. 2018;18(1):18-26.

14. Perez-Flores J, Hernandez-Torres A, Monton F, Nieto A. Health-related quality of life and depressive symptoms in Friedreich ataxia. Qual Life Res. 2020;29(2):413-20.

15. Reetz K, Dogan I, Hohenfeld C, Didszun C, Giunti P, Mariotti C, et al. Nonataxia symptoms in Friedreich Ataxia: Report from the Registry of the European Friedreich’s Ataxia Consortium for Translational Studies (EFACTS). Neurology. 2018;91(10):e917-e30.

16. Chan YC. Aripiprazole treatment for psychosis associated with Friedreich’s ataxia. Gen Hosp Psychiatry. 2005;27(5):372.

17. Ganos C, Schottle D, Zuhlke C, Munchau A. Psychosis complicating Friedreich ataxia. Mov Disord Clin Pract. 2015;2(1):84-5.

18. Salbenblatt MJ, Buzan RD, Dubovsky SL. Risperidone treatment for psychosis in end-stage Friedreich’s ataxia. Am J Psychiatry. 2000;157(2):303.

19. Oruç S, Gulseren G, Kusbeci OY, Yaman M, Geçici O. Quetiapine treatment for psychosis in Friedreich’s ataxia. Klinik Psikofarmakoloji Bulteni – Bulletin of Clinical Psychopharmacology. 2012;22:357-8.

These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.

This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.

It is our expectation that going forward individual topics can be updated in real-time in response to new evidence versus a re-evaluation and update of all topics simultaneously.