Objective: The objective of this study was to evaluate, through polysomnography, the effect of myofunctional therapy in patients with spinocerebellar ataxia type 3.

Background: SCA3/MJD is the most common autosomal dominant-subtype of ataxia worldwide. The disease comprises a wide range of clinical manifestations, which include cerebellarataxia, parkinsonism, dystonia, peripheral neuropathy, pseudoexophthalmos, lowermotor neuron disease, with fasciculations and amyotrophy. Recent clinical data has demonstrated high incidence of sleep complaints in SCA3/MJD. Likewise, sleep complaints have been considered as an important modifier of health-related quality of life in SCAs. The most frequent reported sleep complaints in SCA3/MJD are restless legs syndrome (RLS), REM sleep behavior disorder (RBD), excessive daytime sleepiness (EDS), sleep apnea, and insomnia. These sleep disorders might be a frequent non-motor symptom in SCA3/MJD. However, studies evaluating sleep parameters after speech therapy in patients with ataxia are scarce.

Method: Prospective and longitudinal study with 10 individuals, aged between 26 and 64 years, with diagnosis of  SCA3/MJD confirmed by genetic test or individual with ataxia, in follow-up at the General and Ataxia Neuroscience outpatient clinic of Universidade Federal de São Paulo/UNIFESP, with approval at the Ethics and Research Committee of the UNIFESP (CEP 1206/2016). All patients underwent evaluations before and after the three months of the study. Evaluations included the Epworth Sleepiness Scale (ESS), polysomnography, and a myofunctional evaluation.

Results: The study sample comprised 10 patients who had a mean age of 48.8±12.9 years, a body mass index of 24±5.7 kg/m2. The myofunctional therapy group showed decreased AHI (pre 14.1±13.3 and after therapy 7.1±6.3 p<0.001) and snoring high to low intensity. Table 1 and 2.The myofunctional therapy group also showed improvements in tongue and soft palate muscle strength. However, there was not a significant improvement in ESS (pre 15.6±3.4 and after therapy 13.7±5.6 p>0.05).

Conclusion: The muscular exercise program for the oropharynx of individuals with MJD proposed in the present study was able to reduce the frequency and intensity of snoring, and the severity of apnea, and may be considered an alternative treatment to improve sleep and quality of life in appropriately selected patients with MJD with OSA.

References: Darley FL, Aronson AE, Brown JR. Differential diagnostic patterns of dysarthria. J Speech Hear Res.1969;12:246-269. Duffy JR. Motor Speech disorders. Substrates, differential diagnosis, and management. St Louis: Mosby, 1995. Crary MA, Mann GD, Groher ME. Initial psychometric assessment of a functional oral intake scale for dysphagia in stroke patients. Arch Phys MedRehabil. 2005; 86(8):1516-20.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/effect-of-speech-therapy-in-the-sleep-of-patients-with-machado-joseph-disease-mjd-or-spinocerebellar-ataxia-type-3-sca3/