Clinical Management Guidelines for Friedreich Ataxia (FRDA)

Topic 14.3. Management of anxiety 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.

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.


14.3 Management of anxiety in Friedreich ataxia

Caroline Spencer, Alexandra Durr, George Wilmot, Susan Perlman and Louise Corben
Whilst anxiety has not often been explicitly studied in individuals with FRDA, it appears to not be as common as depression. In a large cohort (n=650) of individuals with FRDA, 20 (3.1%) reported experiencing anxiety (15). Importantly, 18 of those 20 were in the typical onset group, which had a prevalence of 3.3% (15). The clinical experience of the authors indicates there is no difference in response to anti-anxiety medication in individuals with FRDA, compared to those without FRDA. There have not been any studies in FRDA, but if anti-anxiety medication is efficacious as in the general population, then the desirable effect on individuals who are anxious would be large.

It is important to make sure individuals with FRDA are screened and clinicians are alert to the possibility of anxiety. In addition, clinicians should explore what will work best for an individual with FRDA, particularly in the context of other aspects of their lives, such as accessibility requirements of face-to-face counseling and time constraints for work/study/family. The clinician may need to consider treating symptoms of anxiety with both counseling and medication.


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Please note: Recommendations 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. Best Practice Statements are commonly accepted practices, as such formal rating of the quality of evidence by the GRADE process is not indicated. In addition if recommendations from the 2014 guidelines were deemed still relevant, although unable to undergo the scrutiny from a GRADE framework, they were also included as best practice statements.
Anti-anxiety medication

QUESTION: Should anti-anxiety medication versus none be used for anxiety in individuals with Friedreich ataxia?

STRENGTH OF RECOMMENDATION:
LEVEL OF EVIDENCE: ⨁◯◯◯

RECOMMENDATION: We conditionally recommend the use of anti-anxiety medication in individuals with Friedreich ataxia who present with symptoms of anxiety.

JUSTIFICATION: Cohort studies indicate the presence of anxiety symptoms in individuals with Friedreich ataxia. Whilst there is no published evidence in Friedreich ataxia, if treatment is efficacious then the desirable effect on individuals who are anxious would be large. Clinical experience of the authors indicates there is no difference in response to medication in individuals with Friedreich ataxia, when they accept the use of medication, compared to those without Friedreich ataxia. It is important to make sure individuals with Friedreich ataxia are screened and clinicians are alert to the possibility of anxiety.

SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety and should be implemented according to an assessment of the severity of anxiety. Balance of side-effects versus efficacy needs to be taken into account when considering treatment options.

Evidence to Recommendation Table PDF
Counseling for anxiety

QUESTION: Should counseling or therapy versus none or anti-anxiety prescription be used for anxiety in individuals with Friedreich ataxia?

STRENGTH OF RECOMMENDATION: ↑↑
LEVEL OF EVIDENCE: ⨁◯◯◯

RECOMMENDATION: We recommend counseling or therapy over no counseling for individuals with Friedreich ataxia who present with symptoms of anxiety.

JUSTIFICATION: While there is no published evidence in Friedreich ataxia, if counseling is efficacious then the desirable effect on individuals who are anxious would be large. It is important clinicians explore what will work best for an individual with Friedreich ataxia, particularly in the context of other aspects of their lives (accessibility requirements of face-to-face counseling, time constraints for work/study/family, etc.). The clinician may need to consider treating symptoms of anxiety with both counseling and medication.

SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety. It may be difficult for a person with severe dysarthria to engage in counseling. Consideration should be given to a possible link between visual disturbance and the emergence of anxiety.

Evidence to Recommendation Table PDF
Lifestyle changes for anxiety

QUESTION: Should lifestyle changes (exercise, diet, social activities) versus none or anti-anxiety treatment be used for anxiety patients with Friedreich ataxia?

STRENGTH OF RECOMMENDATION:
LEVEL OF EVIDENCE: ⨁◯◯◯

RECOMMENDATION: We conditionally recommend against lifestyle changes as a primary intervention to treat anxiety in individuals with Friedreich ataxia, favoring anti-anxiety medication or counseling prior to or in conjunction with any lifestyle changes.

JUSTIFICATION: There is a lack of research on the effectiveness of lifestyle changes in treating anxiety in individuals with Friedreich ataxia. Medication and/or counseling are likely to be more efficacious.

SUBGROUP CONSIDERATION: This recommendation is for individuals with Friedreich ataxia and symptoms of anxiety.

Evidence to Recommendation Table PDF

Lay summary of clinical recommendations for anxiety in Friedreich ataxia

Individuals with Friedreich ataxia often report mental health concerns. These issues can vary in nature throughout the person’s life, and often include anxiety. Mental health concerns can affect physical, emotional, and social wellbeing.

Why these recommendations?

There are various strategies that may help a person with Friedreich ataxia experiencing anxiety.

Medication

Due to a lack of studies, there is no direct evidence showing the benefits of anti-anxiety medication for treating anxiety in individuals with Friedreich ataxia. However, if medication is effective for treating anxiety symptoms, the benefit to the individual would be large. Ways to limit undesirable side effects (such as dizziness or worsening balance), which would likely have a greater impact on individuals with Friedreich ataxia than other people, should be considered.

Counselling

While there is no direct evidence for the benefits of counselling in individuals with Friedreich ataxia who have anxiety, we recommend counselling to treat these conditions. If counselling is effective, the benefit would be large and there are unlikely to be any undesirable effects.

Lifestyle changes

There is little research or clinical experience indicating that lifestyle changes will improve anxiety. Thus, we recommend other interventions, such as medication or counselling, over lifestyle changes for individuals experiencing anxiety.

What does this mean for you as a person living with Friedreich ataxia or caring for someone living with Friedreich ataxia?

It is important to talk to your doctor or other healthcare professional if you feel anxiety may be an issue for you or someone you care for. A health professional will assess whether the use of behavioural management, counselling or medication may be appropriate for you.

Who are these recommendations specifically for?

These recommendations are relevant to all individuals with Friedreich ataxia who have symptoms of anxiety. Consideration should be given to treatments that are best suited to the age of the individual, the specific diagnosis and the severity of symptoms.

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.

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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.

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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.

For the rating of the strength of the recommendation, in addition to evidence from studies in FRDA, evidence from like conditions, clinical experience and expert consensus are taken into account when published evidence is not available.

The level of evidence is based on published evidence from studies in FRDA. If there is no published evidence in FRDA, evidence from other like conditions or clinical expertise may have been used to make the recommendation – this is graded as ‘very low’ or in some cases ‘low’ level evidence. See the table below for an explanation of the symbols used to grade recommendations.

Strength of recommendation Symbol Level of evidence Symbol
Strong for intervention ↑↑ High ⨁⨁⨁⨁
Conditional for intervention Moderate ⨁⨁⨁◯
Neither intervention nor comparison Low ⨁⨁◯◯
Conditional against intervention Very low ⨁◯◯◯
Strong against intervention ↓↓
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