Dysarthria in hereditary spastic paraplegia type 4
Lais Alves Jacinto-Scudeiro, Rui Rothe-Neves, Vanessa Brzoskowski dos Santos, Gustavo Dariva Machado, Daniela Burguêz, Marina Martins Pereira Padovani, Annelise Ayres, Rafaela Soares Rech, Carelis González-Salazar, Marcondes Cavalcante França, Jonas Alex Morales Saute, Maira Rozenfeld Olchik
ARTIGO
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Agradecimentos: The authors thank all the families who agreed to participate in the present study. The authors also thank Prof. Dr. Leonardo Tartaruga, and to Valéria Feijó Martins and Ana Paula Janner Zanardi for their support in allocating patients during the assessment phase
Abstract: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data. METHODS: Cross-sectional study was carried out in two university hospitals in Brazil. Two groups participated in the study: the case group (n = 28) with...
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Abstract: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data. METHODS: Cross-sectional study was carried out in two university hospitals in Brazil. Two groups participated in the study: the case group (n = 28) with a confirmed genetic diagnosis for SPG4 and a control group (n = 17) matched for sex and age. The speech assessment of both groups included: speech task recording, acoustic analysis, and auditory-perceptual analysis. In addition, disease severity was assessed with the Spastic Paraplegia Rating Scale (SPRS). RESULTS: In the auditory-perceptual analysis, 53.5% (n = 15) of individuals with SPG4 were dysarthric, with mild to moderate changes in the subsystems of phonation and articulation. On acoustic analysis, SPG4 subjects' performances were worse in measurements related to breathing (maximum phonation time) and articulation (speech rate, articulation rate). The articulation variables (speech rate, articulation rate) are related to the age of onset of the first motor symptom. CONCLUSION: Dysarthria in SPG4 is frequent and mild, and it did not evolve in conjunction with more advanced motor diseases. This data suggest that diagnosed patients should be screened and referred for speech therapy evaluation and those pathophysiological mechanisms of speech involvement may differ from the length-dependent degeneration of the corticospinal tract
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CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQ
460941/2014-3
COORDENAÇÃO DE APERFEIÇOAMENTO DE PESSOAL DE NÍVEL SUPERIOR - CAPES
001
CONSELHO NACIONAL DE DESENVOLVIMENTO CIENTÍFICO E TECNOLÓGICO - CNPQ
316036/2021-8
FUNDAÇÃO DE AMPARO À PESQUISA DO ESTADO DO RIO GRANDE DO SUL - FAPERGS
Aberto
Dysarthria in hereditary spastic paraplegia type 4
Lais Alves Jacinto-Scudeiro, Rui Rothe-Neves, Vanessa Brzoskowski dos Santos, Gustavo Dariva Machado, Daniela Burguêz, Marina Martins Pereira Padovani, Annelise Ayres, Rafaela Soares Rech, Carelis González-Salazar, Marcondes Cavalcante França, Jonas Alex Morales Saute, Maira Rozenfeld Olchik
Dysarthria in hereditary spastic paraplegia type 4
Lais Alves Jacinto-Scudeiro, Rui Rothe-Neves, Vanessa Brzoskowski dos Santos, Gustavo Dariva Machado, Daniela Burguêz, Marina Martins Pereira Padovani, Annelise Ayres, Rafaela Soares Rech, Carelis González-Salazar, Marcondes Cavalcante França, Jonas Alex Morales Saute, Maira Rozenfeld Olchik
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