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Published in Henry J. Woodford, Essential Geriatrics, 2022
Electromyogram (EMG) testing is likely to show muscle denervation with preserved velocities when there is LMN involvement. Brain or spine imaging is likely to appear normal. Genetic testing can be considered for people with a family history. There is no curative treatment. Riluzole, a glutamate antagonist, improves survival at 12 months, but has no benefit on muscle strength. The median survival increase is around three to six months.65 Speech and language therapy can help with dysarthria and dysphagia. Tube feeding may be appropriate for selected people with dysphagia. Non-invasive ventilation can prolong survival in appropriate patients. Analgesia may be necessary for joint pains. Spasticity may be present. Advance care planning and palliative care are important aspects of management.
Dysarthria
Published in Margaret Walshe, Nick Miller, Clinical Cases in Dysarthria, 2021
Some underlying causes of dysarthria are amenable to pharmacological or surgical interventions that slow, halt or reverse deterioration. Currently, such options remain restricted. Even then benefits may be time-limited and/or come with negative side effects, individuals need to weigh against possible advantages. The holy grail of halting, reversing or even preventing decline in the first place is the focus of widespread research. Thus, for most people with dysarthria, behavioural management by the SLT/SLP remains the main avenue to tackle their communication issues.
Sandhoff disease/GM2 gangliosidosis/deficiency of Hex A and Hex B subunit deficiency
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
Adult-onset patients may have psychiatric symptoms [14]. They have included confusion, emotional lability and intermittent psychosis. Decline in cognitive function was found in 44 percent of patients. Ataxia and an apparent spinocerebellar picture may be evident [10–15]. Dysarthria may be severe. The disorder may be very slowly progressive. Sensory neuropathy was a predominant finding in one family [15]. In late-onset patients, typical membranous cytoplasmic bodies have been reported in the myenteric plexus [16].
Interaction between people with dysarthria and speech recognition systems: A review
Published in Assistive Technology, 2023
Aisha Jaddoh, Fernando Loizides, Omer Rana
However, people with speech impairments – specifically, those with moderate to severe dysarthria, a neurological motor speech disorder that results in speech difficulties, poor speech intelligibility, difficulty coordinating breaths and speech and inconsistency in the production of the same word over multiple trials – may have difficulty using ASR technologies. Dysarthria could be caused by different diseases, for example, multiple sclerosis or motor neuron disease, or occur after strokes and brain injuries. Research on the interactions between people with dysarthria and speech technologies has been recognized since 1985 (Fried-Oken, 1985), with several studies examining speech intelligibility’s contribution to ASR performance. Ferrier et al. (1995) examined the correlation between speaker intelligibility and recognition success, finding that intelligibility has a strong correlation with ASR performance. Thomas-Stonell et al. (1998), Rosen and Yampolsky (2000), and Young and Mihailidis (2010)
Quantifying articulatory impairments in neurodegenerative motor diseases: A scoping review and meta-analysis of interpretable acoustic features
Published in International Journal of Speech-Language Pathology, 2023
Hannah P. Rowe, Sanjana Shellikeri, Yana Yunusova, Karen V. Chenausky, Jordan R. Green
Neurodegenerative motor diseases (NMDs) are a heterogeneous group of diseases characterised by progressive neuronal loss in the central and peripheral nervous systems, affecting motor function (Duffy, 2013). Dysarthria, which is a speech motor disorder resulting from loss of articulator muscle strength or control, is a common symptom of NMDs (Duffy, 2013), and the consequent communication disturbances significantly impact patient quality of life (Hartelius, Elmberg, Holm, Lovberg, & Nikolaidis, 2008). The type and severity of dysarthria is, therefore, often examined in the assessment and differential diagnosis of neurologic disorders (Duffy, 2013; Kent, Kent, Weismer, & Duffy, 2000). Furthermore, a growing number of studies suggest that speech impairments may be among the most informative motor abnormalities for detecting the onset of neurological dysfunction or tracking the rate of disease progression (Robin et al., 2020). Exploring the efficacy of candidate speech acoustic biomarkers has thus become an active area of research for the purposes of improving early detection, disease classification, and progress monitoring during clinical trials (Carmichael, 2014; Gunduz, 2019; Gutz, Wang, Yunusova, & Green, 2019; Orozco-Arroyave et al., 2016; Tsanas, Little, McSharry, Spielman, & Ramig, 2012; Wang, Kothalkar, Cao, & Heitzman, 2016).
Speech intelligibility of Parkinson’s disease patients evaluated by different groups of healthcare professionals and naïve listeners
Published in Logopedics Phoniatrics Vocology, 2021
Joana Carvalho, Rita Cardoso, Isabel Guimarães, Joaquim J. Ferreira
In this study the primary aim was on the listeners and their capacity to understand PD patients’ speech. The main hypothesis was that professional experienced listeners (Neurologists and Speech and Language Therapists (SLTs)) would have different hit percentages in speech intelligibility than naïve listeners (PD patients, PD relatives, and General Population, GP). Within the naïve speakers those with PD speech familiarity are also expected to have different hit percentages than those without PD speech familiarity. If intelligibility varies within different groups of listeners it is important to take all of them into account when intelligibility is being assessed. Another aim is to understand the dysarthria severity effect in speech intelligibility. Given research suggesting that speech dimensions deteriorate in the course of the PD disease it was hypothesized that severe dysarthria would yield worst speech intelligibility than mild and moderate dysarthria severities. Understanding the speech intelligibility in the different levels of dysarthria may provide specific therapeutic clues to improve the PD patients’ communication.