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CC BY 4.0 · Arq Neuropsiquiatr 2022; 80(10): 1017-1025
DOI: 10.1055/s-0042-1755203
Original Article

Effect of speech therapy on quality of life in patients with spinocerebelar ataxia type 3

Efeito da terapia fonoaudiológica na qualidade de vida de pacientes com ataxia espinocerebelar tipo 3

Authors

  • Giovana Diaféria 

    1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.

  • Silvana Bommarito 

    2   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Fonoaudiologia, São Paulo SP, Brazil.

  • Pedro Braga Neto 

    3   Universidade Federal do Ceará, Serviço de Neurologia e Neurocirurgia, Fortaleza CE, Brazil.

  • Sung Woo Park 

    4   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

  • Marina Padovani 

    5   Faculdade de Ciências Médicas da Santa Casa, Departamento de Fonoaudiologia, São Paulo SP, Brazil.

  • Fernanda Haddad 

    4   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

  • Leonardo Haddad 

    4   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, São Paulo SP, Brazil.

  • Mariana Callil Voos 

    6   Universidade Pontífice Católica de São Paulo, Faculdade de Ciências Humanas e da Saúde, São Paulo SP, Brazil.

  • Hsin Fen Chien 

    7   Universidade de São Paulo, Faculdade de Medicina de São Paulo, Departamento de Ortopedia e Traumatologia, São Paulo SP, Brazil.

  • José Luiz Pedroso 

    1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.

  • Orlando Barsottini 

    1   Universidade Federal de São Paulo, Escola Paulista de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.
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Abstract

Background Individuals with spinocerebellar ataxia type 3 (SCA3) present communication and swallowing disorders, and consequent deterioration in quality of life (QOL).

Objective To evaluate the impact of a speech therapy rehabilitation program on the 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 International Cooperative Ataxia Rating Scale scores were 32.4 ± 20.2, and the Scale for the Assessment and Rating of Ataxia scores were 11.8 ± 8.0. The intervention consisted of a 12-session speech therapy rehabilitation program with oral, pharyngeal, and laryngeal strengthening exercises—the so-called ATAXIA–Myofunctional Orofacial and Vocal Therapy (A-MOVT). They all were submitted to pre- and postintervention evaluations using the World Health Organization's Quality of Life (WHOQOL-BREF) assessment, as well as the Living with Dysarthria (LwD), Quality of Life in Swallowing Disorders (SWAL-QOL), and Food Assessment Tool (EAT-10).

Results The study sample consisted of 48 patients with SCA3 (STG = 25; CG = 23), mean age was 47.1 ± 11.4 years; mean age at symptom onset was 36.9 ± 11.3 years; disease duration was 11.9 ± 13.3 years. After the 3-month intervention, there were significant changes in the QOL in the STG compared with the CG, when assessed by the LwD (179.12 ± 62.55 vs. 129.88 ± 51.42, p < 0.001), SWAL-QOL (869.43 ± 153.63 vs. 911.60 ± 130.90, p = 0.010), and EAT-10 (5.16 ± 7.55 vs. 2.08 ± 3.85, p = 0.018).

Conclusions Patients with SCA3 should receive continuous speech therapy as part of the A-MOVT program, because therapy helps to improve difficulty swallowing and dysarthria.

Resumo

Antecedentes Indivíduos com ataxia espinocerebelar tipo 3 (AEC3) apresentam distúrbios da comunicação e deterioração da deglutição e, consequentemente, na qualidade de vida (QV).

Objetivo Avaliar o impacto de um programa de reabilitação fonoaudiológica na QV em pacientes com AEC3.

Métodos Todos os participantes foram alocados aleatoriamente em dois grupos, um grupo intervenção que recebeu terapia fonoaudiológica (GTF) e um grupo controle (GC). As pontuações das escalas: International Cooperative Ataxia Rating Scale (ICARS) foram 32,4 ± 20,2 e da Scale for the Assessment and Rating of Ataxia (SARA) foram 11,8 ± 8,0. A intervenção consistiu em um programa de reabilitação fonoaudiológica de 12 sessões composto por exercícios de fortalecimento oral, faríngeo e laríngeo - denominados ATAXIA - Terapia Miofuncional Orofacial e Vocal (A-TMOV). Todos foram submetidos a avaliações pré e pós-intervenção por meio dos protocolos World Health Organization's Quality of Life (WHOQOL-BREF), Vivendo com Disartria (VcD), Quality of Life in Swallowing Disorders (SWAL-QOL) e Food Assessment Tool (EAT-10).

Resultados A amostra foi composta por 48 pacientes com AEC3 (25 no GTF e 23 no GC), média de idade 47,1 ± 11,4anos; média de idade de início dos sintomas 36,9 ± 11,3anos; duração da doença 11,9 ± 13,3anos. Após intervenção de três meses, houve mudanças significativas na QV no GTF em comparação com o GC quando avaliado pelo VcD (179,12 ± 62,55 versus129,88 ± 51,42, p < 0,001), SWAL-QOL (869,43 ± 153,63 versus 911,60 ± 130,90, p = 0,010), EAT-10 (5,16 ± 7,55 versus 2,08 ± 3,85, p = 0,018).

Conclusões Pacientes com AEC3 devem receber terapia fonoaudiológica contínua como parte do programa A-TMOV, pois a terapia ajuda a melhorar a dificuldade de deglutição e a disartria.

Keywords

Machado-Joseph Disease - Speech Therapy - Deglutition Disorders - Dysarthria - Quality of Life

Palavras-chave

Doença de Machado-Joseph - Fonoterapia - Transtornos de Deglutição - Disartria - Qualidade de Vida

Authors' Contributions

GD: conception, execution, and organization of patients' evaluation, conception and execution of speech therapy, writing, review, and critique of the manuscript; SB: conception, organization of patients' evaluation, conception and execution of speech therapy, writing and review of the manuscript; PBN: conception and organization of patient's evaluation, review and critique of the manuscript; SP: conception, execution, and organization of patient's evaluation, review and critique of the manuscript; MP: conception and organization of patient's evaluation, conception of speech therapy, review and critique of the manuscript; FH: organization and execution of patients' evaluation, writing, review, and critique of the manuscript; LH: organization and execution of patients' evaluation, review and critique of the manuscript; MCV: conception and organization of patients' evaluation, review and critique of the manuscript; HFC: conception and organization of patients' evaluation, writing, review, and critique of the manuscript; JLP, OGPB: conception, organization, and execution of patients' evaluation, writing, review, and critique of the manuscript.




Publication History

Received: 13 September 2021

Accepted: 01 December 2021

Article published online:
19 December 2022

© 2022. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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