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Abstract
Objectives: We sought to document the prevalence and nature of sleep-disordered breathing (SDB) in individuals with Friedreich ataxia (FRDA) as well as establish the relationship, if any, between SDB and clinical parameters of FRDA.
Methods: Eighty-two individuals with FRDA were administered the Epworth Sleepiness Scale on an annual basis for up to 3 years. Individuals were referred for a sleep study if they had an Epworth Sleepiness Scale score >8 or had clinical symptoms suggestive of SDB.
Results: From this cohort, 21 individuals underwent a sleep study and 17 were diagnosed with obstructive sleep apnea syndrome, giving a minimum prevalence of 21%, which is greater than that found in the general population (3%–7%). Moreover, the presence of obstructive sleep apnea was significantly correlated with the duration of disease and clinical severity of FRDA.
Conclusion: It is recommended that individuals with FRDA undergo regular screening for obstructive sleep apnea to identify the need for a sleep study and subsequent treatment if SDB is diagnosed.
GLOSSARY
- BMI=
- body mass index;
- CSA=
- central sleep apnea;
- ESS=
- Epworth Sleepiness Scale;
- FARS=
- Friedreich Ataxia Rating Scale;
- FRDA=
- Friedreich ataxia;
- ODI=
- Oxygen Desaturation Index;
- OSA=
- obstructive sleep apnea;
- OSAS=
- obstructive sleep apnea syndrome;
- RDI=
- Respiratory Disturbance Index;
- SDB=
- sleep-disordered breathing
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at www.neurology.org
- Received December 19, 2012.
- Accepted in final form March 13, 2013.
- © 2013 American Academy of Neurology
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