Speech therapy evaluation and treatment in patients with Ataxia
- Conditions
- DysarthriaDysphagiaMachado-Joseph Disease
- Registration Number
- RBR-35dk2fc
- Lead Sponsor
- Silvana Bommarito Monteiro
- Brief Summary
Spinocerebellar ataxia type 3 (SCA3) is a degenerative disease that is inherited in an autosomal dominant pattern. It is caused by a mutation located on the chromosome 14q resulting in abnormal CAG triplet repeats. It affects different population groups with an estimated prevalence of 1:100,000 in Brazil. This disease usually manifests between 30 and 50 years of age and first symptoms include ataxia, gait disturbance and abnormal ocular motricity. The degenerative process underlying SCA3 affects many different regions and/or functions of the central nervous system (CNS) and the peripheral nervous system (PNS) including areas and pathways that are involved in motor speech and swallowing. With progression of the disease, individuals with SCA3 exhibit communication and swallowing deterioration. Speech-language interventions for speech, voice and swallowing problems are important because patients with difficulty swallowing are at risk of repeated aspiration and dysarthria affects normal socialization. Objectives: To evaluate the impact of a speech therapy rehabilitation program on phonoarticulation, voice, swallowing and quality of life (QoL) of patients with SCA3. Methods: All participants were randomly assigned to two groups, an intervention group receiving speech therapy (STG) and a control group (CG). The intervention comprised a 12-session speech therapy rehabilitation program consisting of oral, pharyngeal and laryngeal strengthening exercises—so-called ATAXIA – Myofunctional Orofacial and Vocal Therapy (A-MOVT). All participants underwent pre- and post-intervention clinical evaluations using a phonoarticulation assessment tool developed by the Mayo Clinic; nasofibrolaryngoscopy; videoendoscopic swallowing study; and orofacial motricity assessment based on Expanded Protocol of Orofacial Myofunctional Evaluation with Scores (OMES). They were also assessed by four different QoL instruments: The World Health Organization's Quality of Life (WHOQoL-Bref); Living with Dysarthria, (LwD); Quality of Life in Swallowing Disorders (SWAL-QoL); and EAT-10 Food Assessment Tool. Results: The study sample included 48 patients with SCA3 (STG = 25; CG = 23), 33 (69%) females and 15 (31%) males; mean age 47.1±11.4 years; mean age at symptom onset 36.9±11.3 years; and disease duration 11.9±13.3 years. The International Cooperative Ataxia Rating Scale (ICARS) scores were 32.4±20.2 and the Scale for the Assessment and Rating of Ataxia (SARA) scores were 11.8±8.0. At the end of the three-month intervention, the STG showed significant improvements in dysphagia (0.56±0.87 [pre-] vs. 0.00±0.00 [post-]; p<0.001); dysarthria (1.92±0.27 [pre-] vs. 0.84±0.62 [post-], p<0.001); and orofacial motricity (153.00±16.12 [pre-] vs. 205.44±27.55 [post-], p<0.001). There were significant changes in the QoL in the STG compared to the CG when assessed by the LwD (179.12±62.55 vs. 129.88±51.42, p<0.001), SWAL-QoL (79.04±13.97 vs. 82.87±11.91, p=0.010) and EAT-10 (5.16±7.55 vs. 2.08±3.85, p=0.018) and there was an additional effect of speech therapy on clinical parameters regarding the Epworth sleepiness scale (before 12.30 ± 5.5 and after 10.15 ± 6.64, p <0.001) and the snoring intensity (before 1.54 ± 1.07 and after 1.15 ± 1.05, p <0.001) and polysomnographic as to the reduction of the Hypopnea Apnea Index (AHI pre 16.57 ± 20.58 vs. after 11.50 ± 10.98, p = 0.013, sleep efficiency (pre 73.52 ± 10.69 vs. after 75.43 ± 9.26, p <0.001, Arousal index 22.22 ± 12.72 vs. 20.88 ± 5.57, pp <0.001), minimal oxyhemoglobin saturation (SpO2 pre 86.62 ± 7.59 and after SpO2 88.00 ± 7.04, p <0.001). Conclusions: Patients with SCA3 should receive continuous speech therapy as part of the A-MOVT program since rehabilitation therapy improves difficulty swallowing and dysarthria.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Recruitment completed
- Sex
- Not specified
- Target Recruitment
- Not specified
Patients aged between 18 and 70 years of both sexes were included; diagnosed with Spinocerebellar Ataxia Type 3; patients who had not previously undergone speech therapy; with complaints related to voice or swallowing or with associated voice and swallowing complaints
Patients who did not present an exclusive neurological diagnosis of Type 3 Spinocerebellar Ataxia were excluded; who had previously undergone speech therapy; patients with cognitive decline or associated serious or decompensated psychiatric disorders; non-collaborative patients or those with a low level of education; illiterate or with incomplete elementary education that would make it impossible to apply the questionnaires and understand the guidelines; with the presence of marked craniofacial or upper airway anatomical changes; grade III nasal septum deviation and grade III and IV palatine tonsils
Study & Design
- Study Type
- Intervention
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method
- Secondary Outcome Measures
Name Time Method