• Clinics · Jan 2023

    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, JuniorMarcondes Cavalcante FrançaMCFDepartment of Neurology, Universidade Estadual de Campinas, São Paulo, SP, Brazil., SauteJonas Alex MoralesJAMPostgraduate Program in Medicine, Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil; Internal Medicine , and Maira Rozenfeld Olchik.
    • Postgraduate Program in Medicine, Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
    • Clinics (Sao Paulo). 2023 Jan 1; 78: 100128100128.

    ObjectiveTo describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data.MethodsCross-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).ResultsIn 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.ConclusionDysarthria 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.Copyright © 2022 HCFMUSP. Published by Elsevier España, S.L.U. All rights reserved.

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