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Abstract
Although speech declines rapidly in some individuals with amyotrophic lateral sclerosis (ALS), longitudinal changes in speech have rarely been characterized. The study objectives were to model the rate of decline in speaking rate and speech intelligibility as a function of disease onset site, sex, and age at onset in 166 individuals with ALS; and estimate time to speech loss from symptom onset. We also examined the association between clinical (speaking rate/intelligibility) measures and patient-reported measures of ALS progression (ALSFRS-R). Speech measures declined faster in the bulbar-onset group than in the spinal-onset group. The rate of decline was not significantly affected by sex and age. Functional speech was still maintained at 60 months since disease onset for most patients with spinal onset. However, the time to speech loss was 23 months based on speaking rate < 120 (w/m) and 32 months based on speech intelligibility < 85% in individuals with ALS-bulbar onset. Speech measures were more responsive to functional decline than were the patient-reported measures. The findings of this study will inform future work directed toward improving speech prognosis in ALS, which is critical for determining the appropriate timing of interventions, providing appropriate counseling for patients, and evaluating functional changes during clinical trials.
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1 MGH Institute of Health Professions, Depatment of Communication Sciences and Disorders, Boston, USA (GRID:grid.429502.8) (ISNI:0000 0000 9955 1726); The Athinoula A. Martinos Center for Biomedical Images, MGH, Harvard Medical School, Department of Radiology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X)
2 Rehabilitation Sciences Institute, University of Toronto, Department of Speech-Language Pathology, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938); Hurvitz Brain Sciences Program, Sunnybrook Research Institute, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938); KITE –Toronto Rehabilitation Institute – University Health Network, Toronto, Canada (GRID:grid.231844.8) (ISNI:0000 0004 0474 0428)
3 MGH Institute of Health Professions, Depatment of Communication Sciences and Disorders, Boston, USA (GRID:grid.429502.8) (ISNI:0000 0000 9955 1726)
4 Sean M. Healey and AMG Center for ALS, MGH, Department of Neurology, Boston, USA (GRID:grid.32224.35) (ISNI:0000 0004 0386 9924)
5 University of Toronto, Division of Neurology, Department of Medicine, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938); Sunnybrook Health Sciences Centre, Toronto, Canada (GRID:grid.413104.3) (ISNI:0000 0000 9743 1587)
6 Neuroscience and Mental Health Institute, University of Alberta, Edmonton, Canada (GRID:grid.17089.37) (ISNI:0000 0001 2190 316X); University of Alberta, Division of Neurology, Edmonton, Canada (GRID:grid.17089.37) (ISNI:0000 0001 2190 316X)
7 University of Calgary, Department of Clinical Neurosciences, Hotchkiss Brain Institute, Calgary, Canada (GRID:grid.22072.35) (ISNI:0000 0004 1936 7697)
8 Montreal Neurological Institute & Hospital, Department of Neurology and Neurosurgery, Clinical Research Unit, Montreal, Canada (GRID:grid.416102.0) (ISNI:0000 0004 0646 3639)
9 Centre de Recherche du CHU de Québec - Université Laval, Montreal, Canada (GRID:grid.23856.3a) (ISNI:0000 0004 1936 8390)
10 MGH Institute of Health Professions, Depatment of Communication Sciences and Disorders, Boston, USA (GRID:grid.429502.8) (ISNI:0000 0000 9955 1726); Harvard University, Program in Speech and Hearing Bioscience and Technology, Boston, USA (GRID:grid.38142.3c) (ISNI:000000041936754X)