Speech therapy evaluation recommended
Mild-to-moderate changes in the phonation and articulation subsystems of speech were found in just over half of the 28 study participants with SPG4, with the recommendation for speech therapy evaluation.
Objective: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlate 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.
SOURCE: Clinics (Sao Paulo). 2022 Dec 3;78:100128. doi: 10.1016/j.clinsp.2022.100128. eCollection 2023. PMID: 36473366 Copyright © 2022 HCFMUSP. Published by Elsevier España, S.L.U. All rights reserved.
Dysarthria in hereditary spastic paraplegia type 4
Lais Alves Jacinto-Scudeiro 1 , Rui Rothe-Neves 2 , Vanessa Brzoskowski Dos Santos 1 , Gustavo Dariva Machado 3 , Daniela Burguêz 3 , Marina Martins Pereira Padovani 4 , Annelise Ayres 5 , Rafaela Soares Rech 5 , Carelis González-Salazar 6 , Marcondes Cavalcante França Junior 7 , Jonas Alex Morales Saute 8 , Maira Rozenfeld Olchik 9
1. Postgraduate Program in Medicine, Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
2. Phonetics Laboratory of the Faculty of Letters, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
3. Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.
4. Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, SP, Brazil.
5. Universidade Federal de Ciências da Saúde de Porto Alegre, Porto Alegre, RS, Brazil.
6. Postgraduate Program in Medical Pathophysiology, Universidade Estadual de Campinas, São Paulo, SP, Brazil.
7. Department of Neurology, Universidade Estadual de Campinas, São Paulo, SP, Brazil.
8. Postgraduate 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 Department, Faculdade de Medicina Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
9. Postgraduate 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; Department of Surgery and Orthopedics, Faculdade de Odontologia, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil.
I have SPG4 and I recall in the early years of primary school having speech therapy after school either once or twice a week. Can’t recall for how long and unsure of the speech issue involved (possibly finishing words clearly, may have been slightly slurred). I have not any speech issues since and clearly the therapy was very beneficial. On the basis that I wore shoes out when I was young, and still do, I suspect I have had HSP all my life.
What is the current status of the research on noskapine, and is there any relevant news?
Editor’s note: there has been no further research on Noscapine for the treatment of SPG4 HSP since the decision not to proceed with clinical trials for multiple reasons some time ago.