6.3.R3 Recommendation
Complementary/alternative treatments
- Strength of recommendation:
- Conditional against intervention
- Level of evidence:
- Very low
How the strength and level of evidence are graded
What the strength of a recommendation means
The strength of a recommendation reflects the extent to which a guideline panel is confident that desirable effects of an intervention outweigh undesirable effects, or vice versa, across the range of patients for whom the recommendation is intended.
Strong for (↑↑) or against (↓↓) intervention
For patients: Most individuals in this situation would want the recommended course of action and only a small proportion would not.
For clinicians: Most individuals should receive the recommended course of action.
Conditional for (↑) or against (↓) intervention
For patients: The majority of individuals in this situation would want the suggested course of action, but many would not.
For clinicians: Recognize that different choices will be appropriate for different patients, and that you must help each patient arrive at a management decision consistent with her or his values and preferences.
Neither intervention nor comparison (—)
The panel did not recommend for or against the intervention.
Source: GRADE Handbook, section 6.1, Table 6.1 (GRADE uses “weak” and “conditional” interchangeably).
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 | ↓↓ |
We suggest alternative/complementary treatments should not be used over no treatment/medication/lifestyle/physiotherapy for RLS in Friedreich ataxia.
Question
Should complementary/alternative treatments versus none or medication or lifestyle or physiotherapy be used for individuals with restless legs syndrome (RLS) symptoms with Friedreich ataxia?
Justification
Based on no published evidence on complementary/alternative treatments in Friedreich ataxia, we suggest that regular physical activity, sleep hygiene, regular hours for going to bed, avoiding caffeine before bed and the use of electronic devices in bed may assist in managing RLS. A review of current medication is also suggested, which should focus in particular on selective serotonin reuptake inhibitors (SSRI), tricyclics and metoclopramide.
RLS is a significant problem affecting 44.8% of adults with Friedreich ataxia. Complementary/alternative treatments may have undesirable side-effects for a person with Friedreich ataxia. There is no RCT on the use of complementary/ alternative treatments on RLS in Friedreich ataxia. However, if there are no undesirable side-effects, some people with RLS may find alternative treatments as helpful as medication and without the known side-effects of medication.
It should be borne in mind when recommending any complementary/alternative treatments that they may be expensive and private insurers may not cover the cost for a person with Friedreich ataxia.
Subgroup consideration
This recommendation is for individuals with Friedreich ataxia with symptoms of RLS. Restless legs are more common in those who have sleep disturbances.
Evidence to recommendation table (PDF)
Part of 6.3 Restless legs and/or sleep related periodic limb movements
References (22)
1. Bach JR, Turcios NL, Wang L. Respiratory Complications of Pediatric Neuromuscular Diseases. Pediatr Clin North Am. 2021;68(1):177-91.
2. Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018;17(4):347-61.
3. Buu MC. Respiratory complications, management and treatments for neuromuscular disease in children. Curr Opin Pediatr. 2017;29(3):326-33.
4. Sheehan DW, Birnkrant DJ, Benditt JO, Eagle M, Finder JD, Kissel J, et al. Respiratory management of the patient with Duchenne muscular dystrophy. Pediatrics. 2018;142(Suppl 2):S62-S71.
5. Boentert M, Cao M, Mass D, De Mattia E, Falcier E, Goncalves M, et al. Consensus-based care recommendations for pulmonologists treating adults with myotonic dystrophy type 1. Respiration. 2020;99(4):360-8.
6. Tsou AY, Paulsen EK, Lagedrost SJ, Perlman SL, Mathews KD, Wilmot GR, et al. Mortality in Friedreich ataxia. J Neurol Sci. 2011;307:46-9.
7. Silva IS, Pedrosa R, Azevedo IG, Forbes AM, Fregonezi GA, Dourado Junior ME, et al. Respiratory muscle training in children and adults with neuromuscular disease. Cochrane Database Syst Rev. 2019;9:CD011711.
8. Williamson E, Pederson N, Rawson H, Daniel T. The effect of inspiratory muscle training on Duchenne muscular dystrophy: a meta-analysis. Pediatr Phys Ther. 2019;31(4):323-30.
9. Botez MI, Mayer P, Bellemare F, Couture J. Can we treat respiratory failure in Friedreich ataxia? Arch Neurol. 1997;54(8):1030-3.
10. Manni R, Tartara A, Marchioni E, Piccolo G. Polygraphic sleep patterns in heredoataxia: a study of nine cases. Revue d Electroencephalographie et de Neurophysiologie Clinique. 1986;16(2):117-21.
11. Corben LA, Ho M, Copland J, Tai G, Delatycki MB. Increased prevalence of sleep-disordered breathing in Friedreich ataxia. Neurology. 2013;81(1):46-51.
12. Patterson A, Almeida L, Monari E, et al. Sleep and fatigue in Friedreich ataxia. IARC Meeting; Pisa, Italy 2018.
13. Annane D, Orlikowski D, Chevret S. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev. 2014(12):CD001941.
14. Lynch D. FA Clinical Outcome Measures (FA-COMS) Registry (unpublished data): clinicaltrials.gov; 2017 [Available from: https://clinicaltrials.gov/ct2/show/NCT03090789
15. Ohayon MM, O'Hara R, Vitiello MV. Epidemiology of restless legs syndrome: a synthesis of the literature. Sleep Med Rev. 2012;16(4):283-95.
16. American Academy of Sleep Medicine. International classification of sleep disorders. Westchester, IL: American Academy of Sleep Medicine; 2014.
17. Marino M, Li Y, Rueschman MN, Winkelman JW, Ellenbogen JM, Solet JM, et al. Measuring sleep: accuracy, sensitivity, and specificity of wrist actigraphy compared to polysomnography. Sleep. 2013;36(11):1747-55.
18. Fulda S. The Role of Periodic Limb Movements During Sleep in Restless Legs Syndrome: A Selective Update. Sleep Med Clin. 2015;10(3):241-8, xii.
19. Winkelmann J, Allen RP, Hogl B, Inoue Y, Oertel W, Salminen AV, et al. Treatment of restless legs syndrome: Evidence-based review and implications for clinical practice (Revised 2017). Mov Disord. 2018;33(7):1077-91.
20. Frauscher B, Hering S, Hogl B, Gschliesser V, Ulmer H, Poewe W, et al. Restless legs syndrome in Friedreich ataxia: a polysomnographic study. Mov Disord. 2011;26(2):302-6.
21. Bega D, Malkani R. Alternative treatment of restless legs syndrome: an overview of the evidence for mind-body interventions, lifestyle interventions, and neutraceuticals. Sleep Med. 2016;17:99-105.
22. Trotti LM, Becker LA. Iron for the treatment of restless legs syndrome. Cochrane Database Syst Rev. 2019;1:CD007834.
Disclaimer, intended use and 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.