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Quality of Life since Pre-Ataxic Phases of Spinocerebellar Ataxia Type 3/Machado–Joseph Disease

Bolzan, Gabriela ; Leotti, Vanessa Bielefeldt ; de Oliveira, Camila Maria ; Ecco, Gabriela ; Cappelli, Amanda Henz ; Rocha, Anastacia Guimarães ; Kersting, Nathalia ; Rieck, Mariana ; de Sena, Lucas Schenatto ; Martins, Ana Carolina ; Saraiva-Pereira, Maria-Luiza ; Jardim, Laura Bannach

Cerebellum (London, England), 2022-04, Vol.21 (2), p.297-305 [Periódico revisado por pares]

New York: Springer US

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Título:
Quality of Life since Pre-Ataxic Phases of Spinocerebellar Ataxia Type 3/Machado–Joseph Disease
Autor: Bolzan, Gabriela ; Leotti, Vanessa Bielefeldt ; de Oliveira, Camila Maria ; Ecco, Gabriela ; Cappelli, Amanda Henz ; Rocha, Anastacia Guimarães ; Kersting, Nathalia ; Rieck, Mariana ; de Sena, Lucas Schenatto ; Martins, Ana Carolina ; Saraiva-Pereira, Maria-Luiza ; Jardim, Laura Bannach
Assuntos: Ataxia ; Biomedical and Life Sciences ; Biomedicine ; Clinical trials ; Double-Blind Method ; Health care ; Humans ; Machado-Joseph disease ; Machado-Joseph Disease - genetics ; Neurobiology ; Neurology ; Neurosciences ; Original Article ; Quality of Life ; Spinocerebellar Ataxias - genetics ; Statistical analysis ; Surveys and Questionnaires
É parte de: Cerebellum (London, England), 2022-04, Vol.21 (2), p.297-305
Notas: ObjectType-Article-1
SourceType-Scholarly Journals-1
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Descrição: Although health-related quality of life (HRQoL) has been increasingly valued in healthcare and in clinical trials, there is scarce information about it in spinocerebellar ataxia type 3/Machado–Joseph disease (SCA3/MJD). This study describes the HRQoL results obtained from ataxic SCA3/MJD subjects, and their non-ataxic offspring included in the BIGPRO (Biomarkers and genetic modifiers in a study of presymptomatic and symptomatic SCA3/MJD carriers) study. Demographic data, clinical scales, and HRQoL instruments EQ-5D-3L and SF-36 were collected. Subjects at 50% risk were genotyped in a double-blind manner. The time left until the onset of the disease was estimated for mutation carriers with a SARA < 3 and combined with disease duration of ataxic subjects (TimeToAfterOnset). Analyses were performed using PASW Statistics version 18.0, R version 4.0.0, and G*Power 3.1, and p < 0.05 was considered statistically significant. Twenty-three ataxic carriers, 33 pre-ataxic carriers, and 21 controls were enrolled. Significant differences between ataxic carriers and controls were seen in EQ-VAS, EQ-5D Index, and in some domains of EQ-5D-3L and SF-36. EQ-5D Index showed the best effect size between ataxic and controls (Cohen’s d = 2.423). Stepwise changes were seen in pre-ataxic subjects, although not statistically significant. TimeToAfterOnset correlated with EQ-5D Index, EQ-VAS, and SF-36 Physical functioning, Role Physical, Pain, and General Health. EQ-5D Index and EQ-VAS correlated with clinical scales in the ataxic group. These results suggest that HRQoL worsens among carriers since pre-ataxic stages and that they might encompass the underlying disease process. In this cohort, SF-36 Physical Functioning, SF-36 General health, and especially EQ-5D Index and EQ-VAS were the best HRQoL instruments to be used as ancillary evidence to support biological and social meanings for future interventions.
Editor: New York: Springer US
Idioma: Inglês

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Quality of Life since Pre-Ataxic Phases of Spinocerebellar Ataxia Type 3/Machado–Joseph Disease

Bolzan, Gabriela ; Leotti, Vanessa Bielefeldt ; de Oliveira, Camila Maria ; Ecco, Gabriela ; Cappelli, Amanda Henz ; Rocha, Anastacia Guimarães ; Kersting, Nathalia ; Rieck, Mariana ; de Sena, Lucas Schenatto ; Martins, Ana Carolina ; Saraiva-Pereira, Maria-Luiza ; Jardim, Laura Bannach

New York: Springer US

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