Assessing Oromotor Capacity in ALS: The Effect of a Fixed-Target Task on Lip Biomechanics

This work is licensed under a Creative Commons Attribution 4.0 International License.Alternating motion rate (AMR) is a standard measure often included in neurological examinations to assess orofacial neuromuscular integrity. AMR is typically derived from recordings of patients producing repetitions of a single syllable as fast and clear as possible on one breath. Because the task places high demands on oromotor performance, particularly articulatory speed, AMRs are widely considered to be tests of maximum performance and, therefore, likely to reveal underlying neurologic deficits. Despite decades of widespread use, biomechanical studies have shown that speakers often circumvent the presumed speed challenge of the standard AMR task. Specifically, speakers are likely to manipulate their displacements (movement amplitude) instead of speed because this strategy requires less motor effort. The current study examined the effectiveness of a novel fixed-target paradigm for minimizing the truncation of articulatory excursions and maximizing motor effort. We compared the standard AMR task to that of a fixed-target AMR task and focused specifically on the tasks' potential to detect decrements in lip motor performance in persons with dysarthria due to amyotrophic lateral sclerosis (ALS). Our participants were 14 healthy controls and 17 individuals with ALS. For the standard AMR task, participants were instructed to produce the syllable /bα/ as quickly and accurately as possible on one breath. For the fixed-target AMR task, participants were given the same instructions, but were also required to strike a physical target placed under the jaw during the opening phase of each syllable. Lip kinematic data were obtained using 3D electromagnetic articulography. 16 kinematic features were extracted using an algorithmic approach. Findings revealed that compared to the standard task, the fixed-target AMR task placed increased motor demands on the oromotor system by eliciting larger excursions, faster speeds, and greater spatiotemporal variability. In addition, participants with ALS exhibited limited ability to adapt to the higher articulatory demands of the fixed-target task. Between the two AMR tasks, the maximum speed during the fixed-target task showed a moderate association with the ALSFRS-R bulbar subscore. Employment of both standard and fixed-target AMR tasks is, however, needed for comprehensive assessment of oromotor function and for elucidating profiles of task adaptation

Current status of clinical outcome measures in inclusion body myositis: a systematised review

OBJECTIVES: Sporadic inclusion body myositis (IBM) is a debilitating idiopathic inflammatory myopathy (IIM) which affects hand function, ambulation, and swallowing. There is no approved pharmacological therapy for IBM, and there is a lack of suitable outcome measure to assess the effect of an intervention. The IBM scientific interest group under IMACS reviewed the previously used outcome measures in IBM clinical studies to lay the path for developing a core set of outcome measures in IBM. METHODS: In this systematised review, we have extracted all outcome measures reported in IBM clinical studies to determine what measures were being used and to assess the need for optimising outcome measures in IBM. RESULTS: We found 13 observational studies, 17 open-label clinical trials, and 15 randomised control trials (RCTs) in IBM. Six-minute walk distance, IBM-functional rating scale (IBM-FRS), quantitative muscle testing, manual muscle testing, maximal voluntary isometric contraction testing, and thigh muscle volume measured by MRI were used as primary outcome measures. Twelve different outcome measures of motor function were used in IBM clinical trials. IBM-FRS was the most used measure of functionality. Swallowing function was reported as a secondary outcome measure in only 3 RCTs. CONCLUSIONS: There are inconsistencies in using outcome measures in clinical studies in IBM. The core set measures developed by the IMACS group for other IIMs are not directly applicable to IBM. As a result, there is an unmet need for an IBM-specific core set of measures to facilitate the evaluation of new potential therapeutics for IBM

Comparison of intelligibility measures for adults with Parkinson's disease, Multiple Sclerosis and healthy controls

Purpose: The current study sought to investigate the relationship between two metrics of sentence intelligibility in adults with Parkinson’s Disease (PD), Multiple Sclerosis (MS), and healthy controls. An objective measure of intelligibility, orthographic transcription, and a subjective measure of intelligibility, Visual Analog Scaling (VAS), were the two metrics of intelligibility examined. Areas of interest included 1) comparisons of the pattern of intelligibility change in transcription and VAS, 2) strength of the relationship between these two types of intelligibility measures, and 3) differences in intralistener and interlistener reliability between the two metrics. Methods: 78 speakers and the speech samples reported in Tjaden, Sussman, and Wilding (2014) and Kuo, Tjaden, and Sussman (2014) were used in the current study. The pool of 78 speakers consisted of 32 healthy control speakers, 16 speakers with PD, and 30 speakers with MS. Speakers read Harvard Psychoacoustic Sentences in habitual, clear, fast, loud, and slow conditions. In Tjaden et al. (2014) and Kuo et al. (2014), 50 naive listeners used a VAS on a computer to estimate how much of the speaker’s message was understood (e.g., from ‘didn’t understand anything’ to ‘understand everything’). In the current study, 50 naive listeners heard the same stimuli, but were instructed to type exactly what they heard. Responses were scored to obtain a percentage of key words transcribed correctly for each stimulus. Results from the current study were compared to results from the VAS task studies (Tjaden et al., 2014; Kuo et al., 2014) using descriptive statistics (e.g., mean, standard deviation, etc.), parametric statistics (e.g., multivariate linear model fit to the data in this repeated measured design), correlation analyses (e.g., between the two metrics), and metrics of reliability. Results and Discussion: Results revealed that the pattern of transcription intelligibility scores was very similar to scaled intelligibility derived from VAS. However, transcription scores were higher in magnitude than the VAS scores. In addition, correlation analyses showed the two intelligibility measures were highly correlated. Last, both interlistener and intralistener reliability were marginally higher for the VAS reported in Tjaden et al. (2014) and Kuo et al. (2014) than for the transcription data in the current study. These results suggest that a less time-consuming task, such as the VAS task, may be a viable substitute for a more time-consuming transcription task when documenting intelligibility in a clinical population to obtain an overall metric of severity for tracking disease progression and/or treatment progress

Intelligibility Across a Reading Passage: The Effect of Dysarthria and Cued Speaking Styles

Objective: Reading a passage out loud is a commonly used task in the perceptual assessment of dysarthria. The extent to which perceptual characteristics remain unchanged or stable over the time course of a passage is largely unknown. This study investigated crowdsourced visual analogue scale (VAS) judgments of intelligibility across a reading passage as a function of cued speaking styles commonly used in treatment to maximize intelligibility. Patients and Method: The Hunter passage was read aloud in habitual, slow, loud, and clear speaking styles by 16 speakers with Parkinson’s disease (PD), 30 speakers with multiple sclerosis (MS), and 32 control speakers. VAS judgments of intelligibility from three fragments representing the beginning, middle, and end of the reading passage were obtained from 540 crowdsourced online listeners. Results: Overall passage intelligibility was reduced for the two clinical groups relative to the control group. All speaker groups exhibited intelligibility variation across the reading passage, with trends of increased intelligibility toward the end of the reading passage. For control speakers and speakers with PD, patterns of intelligibility variation across passage reading did not differ with speaking style. For the MS group, intelligibility variation across the passage was dependent on speaking style. Conclusions: The presence of intelligibility variation within a reading passage warrants careful selection of speech materials in research and clinical practice. Results further indicate that the crowdsourced VAS rating paradigm is useful to document intelligibility in a reading passage for different cued speaking styles commonly used in treatment for dysarthria

Intelligibility Across a Reading Passage: The Effect of Dysarthria and Cued Speaking Styles

Objective: Reading a passage out loud is a commonly used task in the perceptual assessment of dysarthria. The extent to which perceptual characteristics remain unchanged or stable over the time course of a passage is largely unknown. This study investigated crowdsourced visual analogue scale (VAS) judgments of intelligibility across a reading passage as a function of cued speaking styles commonly used in treatment to maximize intelligibility. Patients and Method: The Hunter passage was read aloud in habitual, slow, loud, and clear speaking styles by 16 speakers with Parkinson’s disease (PD), 30 speakers with multiple sclerosis (MS), and 32 control speakers. VAS judgments of intelligibility from three fragments representing the beginning, middle, and end of the reading passage were obtained from 540 crowdsourced online listeners. Results: Overall passage intelligibility was reduced for the two clinical groups relative to the control group. All speaker groups exhibited intelligibility variation across the reading passage, with trends of increased intelligibility toward the end of the reading passage. For control speakers and speakers with PD, patterns of intelligibility variation across passage reading did not differ with speaking style. For the MS group, intelligibility variation across the passage was dependent on speaking style. Conclusions: The presence of intelligibility variation within a reading passage warrants careful selection of speech materials in research and clinical practice. Results further indicate that the crowdsourced VAS rating paradigm is useful to document intelligibility in a reading passage for different cued speaking styles commonly used in treatment for dysarthria

No differential effects of subthalamic nucleus vs. globus pallidus deep brain stimulation in Parkinson’s disease: Speech acoustic and perceptual findings

Background: Deep Brain Stimulation (DBS) in the Subthalamic Nucleus (STN) or the Globus Pallidus Interna (GPI) is well-established as a surgical technique for improving global motor function in patients with idiopathic Parkinson’s Disease (PD). Previous research has indicated speech deterioration in more than 30% of patients after STN-DBS implantation, whilst speech outcomes following GPI-DBS have received far less attention. Research comparing speech outcomes for patients with PD receiving STN-DBS and GPI-DBS can inform pre-surgical counseling and assist with clinician and patient decision-making when considering the neural targets selected for DBS-implantation. The aims of this pilot study were (1) to compare perceptual and acoustic speech outcomes for a group of patients with PD receiving bilateral DBS in the STN or the GPI with DBS stimulation both ON and OFF, and (2) examine associations between acoustic and perceptual speech measures and clinical characteristics. Methods: Ten individuals with PD receiving STN-DBS and eight individuals receiving GPI-DBS were audio-recorded reading a passage. Three listeners blinded to neural target and stimulation condition provided perceptual judgments of intelligibility and overall speech severity. Speech acoustic measures were obtained from the recordings. Acoustic and perceptual measures and clinical characteristics were compared for the two neural targets and stimulation conditions. Results: Intelligibility and speech severity were not significantly different across neural target or stimulation conditions. Generally, acoustic measures were also not statistically different for the two neural targets or stimulation conditions. Acoustic measures reflecting more varied speech prosody were associated with improved intelligibility and lessened severity. Convergent correlations were found between UPDRS-III speech scores and perceptual measures of intelligibility and severity. Conclusion: This study reports a systematic comparison of perceptual and acoustic speech outcomes following STN-DBS and GPI-DBS. Statistically significant differences in acoustic measures for the two neural targets were small in magnitude and did not yield group differences in perceptual measures. The absence of robust differences in speech outcomes for the two neural targets has implications for pre-surgical counseling. Results provide preliminary support for reliance on considerations other than speech when selecting the target for DBS in patients with PD