Differential Effects of Velopharyngeal Dysfunction on Speech Intelligibility During Early and Late Stages of Amyotrophic Lateral Sclerosis

Purpose This study describes a phonetic complexity-based approach for speech intelligibility and articulatory precision testing using preliminary data from talkers with amyotrophic lateral sclerosis. Method Eight talkers with amyotrophic lateral sclerosis and 8 healthy controls produced a list of 16 low and high complexity words. Sixty-four listeners judged the samples for intelligibility, and 2 trained listeners completed phoneme-level analysis to determine articulatory precision. To estimate percent intelligibility, listeners orthographically transcribed each word, and the transcriptions were scored as being either accurate or inaccurate. Percent articulatory precision was calculated based on the experienced listeners' judgments of phoneme distortions, deletions, additions, and/or substitutions for each word. Articulation errors were weighted based on the perceived impact on intelligibility to determine word-level precision. Results Between-groups differences in word intelligibility and articulatory precision were significant at lower levels of phonetic complexity as dysarthria severity increased. Specifically, more severely impaired talkers showed significant reductions in word intelligibility and precision at both complexity levels, whereas those with milder speech impairments displayed intelligibility reductions only for more complex words. Articulatory precision was less sensitive to mild dysarthria compared to speech intelligibility for the proposed complexity-based approach. Conclusions Considering phonetic complexity for dysarthria tests could result in more sensitive assessments for detecting and monitoring dysarthria progression.

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Articulatory-to-Acoustic Relations in Talkers With Dysarthria: A First Analysis

Journal of Speech Language and Hearing Research ◽

10.1044/2015_jslhr-s-14-0188 ◽

2015 ◽

Vol 58 (3) ◽

pp. 576-589 ◽

Cited By ~ 20

Author(s):

Antje Mefferd

Keyword(s):

Parkinson’S Disease ◽

Amyotrophic Lateral Sclerosis ◽

Correlation Coefficients ◽

Speaking Rate ◽

Differential Effects ◽

Vowel Contrast ◽

Induced Changes ◽

Tongue Displacement ◽

Lateral Sclerosis ◽

Fast Rates

Purpose The primary purpose of this study was to determine the strength of interspeaker and intraspeaker articulatory-to-acoustic relations of vowel contrast produced by talkers with dysarthria and controls. Methods Six talkers with amyotrophic lateral sclerosis (ALS), six talkers with Parkinson's disease (PD), and 12 controls repeated a sentence at typical, slow, and fast rates. Tongue displacements and acoustic vowel distances were measured to determine articulatory and acoustic vowel contrasts. Results Interspeaker articulatory-to-acoustic relations were strong for talkers with PD and controls but weak for talkers with ALS and controls. Further, predominantly moderate and strong intraspeaker articulatory-to-acoustic relations were found in response to rate modulations; however, correlation coefficients were significantly lower in talkers with ALS than in controls. Conclusions The findings on interspeaker articulatory-to-acoustic relations suggested that the degree of tongue displacement can be accurately inferred from the degree of acoustic vowel contrast in talkers with PD but not in talkers with ALS. Findings on intraspeaker articulatory-to-acoustic relations generally supported the longstanding notion that speaking rate–induced changes in tongue displacement evoke similar changes in acoustic vowel contrast. Differential effects of the pathophysiology on inter- and intraspeaker articulatory-to-acoustic relations are discussed.

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Delivering bad news in amyotrophic lateral sclerosis: Proposal of a specific technique ALS ALLOW

Neurology Clinical Practice ◽

10.1212/cpj.0000000000000957 ◽

2020 ◽

pp. 10.1212/CPJ.0000000000000957

Author(s):

Wesleigh F. Edwards ◽

Sahana Malik ◽

Jonathan Peters ◽

Ivy Chippendale ◽

John Ravits

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Communication Skills ◽

Physician Communication ◽

Emotional Trauma ◽

Specific Technique ◽

Als Patients ◽

Delivering Bad News ◽

Late Stages ◽

Lateral Sclerosis

AbstractPurposeof Review: Physician communication skills are a critical part of care for amyotrophic lateral sclerosis (ALS) patients and caregivers. They shape the development of autonomy and quality of life, and they mitigate emotional trauma. Communication skills are especially critical at two different time points in the course of disease: early when delivering and establishing the diagnosis, and later when clarifying goals of care.Recent Findings:Several techniques for physician communication of difficult information are available, including SPIKES, ABCDE, and BREAKS. These emphasize the physician’s accountability and responsibility for communicating effectively. Formal training in these techniques is limited and their applicability specifically to ALS is inexact.Summary:We propose an ALS-specific technique which we call ALS ALLOW to guide physicians in conducting difficult communications with ALS patients and caregivers to develop their understanding, establish autonomy, set goals, and mitigate emotional trauma. The techniques are useful in discussions both early and late stages in disease.

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Predicting Early Bulbar Decline in Amyotrophic Lateral Sclerosis: A Speech Subsystem Approach

Behavioural Neurology ◽

10.1155/2015/183027 ◽

2015 ◽

Vol 2015 ◽

pp. 1-11 ◽

Cited By ~ 39

Author(s):

Panying Rong ◽

Yana Yunusova ◽

Jun Wang ◽

Jordan R. Green

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Speech Intelligibility ◽

Disease Onset ◽

Principal Component ◽

Speaking Rate ◽

Linear Mixed Effects ◽

Data Mining Approach ◽

Kaplan Meier ◽

Bulbar Dysfunction ◽

Lateral Sclerosis

Purpose. To develop a predictive model of speech loss in persons with amyotrophic lateral sclerosis (ALS) based on measures of respiratory, phonatory, articulatory, and resonatory functions that were selected using a data-mining approach.Method. Physiologic speech subsystem (respiratory, phonatory, articulatory, and resonatory) functions were evaluated longitudinally in 66 individuals with ALS using multiple instrumentation approaches including acoustic, aerodynamic, nasometeric, and kinematic. The instrumental measures of the subsystem functions were subjected to a principal component analysis and linear mixed effects models to derive a set of comprehensive predictors of bulbar dysfunction. These subsystem predictors were subjected to a Kaplan-Meier analysis to estimate the time until speech loss.Results. For a majority of participants, speech subsystem decline was detectible prior to declines in speech intelligibility and speaking rate. Among all subsystems, the articulatory and phonatory predictors were most responsive to early bulbar deterioration; and the resonatory and respiratory predictors were as responsive to bulbar decline as was speaking rate.Conclusions. The articulatory and phonatory predictors are sensitive indicators of early bulbar decline due to ALS, which has implications for predicting disease onset and progression and clinical management of ALS.

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Articulatory Kinematic Characteristics Across the Dysarthria Severity Spectrum in Individuals With Amyotrophic Lateral Sclerosis

American Journal of Speech-Language Pathology ◽

10.1044/2017_ajslp-16-0230 ◽

2018 ◽

Vol 27 (1) ◽

pp. 258-269 ◽

Cited By ~ 9

Author(s):

Jimin Lee ◽

Michael Bell ◽

Zachary Simmons

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Speech Intelligibility ◽

Speaking Rate ◽

Tongue Movement ◽

Jaw Movement ◽

Movement Trajectories ◽

Electromagnetic Articulography ◽

Kinematic Characteristics ◽

Temporal Measures ◽

Lateral Sclerosis

Purpose The current study investigated whether articulatory kinematic patterns can be extrapolated across the spectrum of dysarthria severity in individuals with amyotrophic lateral sclerosis (ALS). Method Temporal and spatial articulatory kinematic data were collected using electromagnetic articulography from 14 individuals with dysarthria secondary to ALS and 6 typically aging speakers. Speech intelligibility and speaking rate were used as indices of severity. Results Temporal measures (duration, speed of articulators) were significantly correlated with both indices of severity. In speakers with dysarthria, spatial measures were not correlated with severity except in 3 measures: tongue movement displacement was more reduced in the anterior–posterior dimension; jaw movement distance was greater in the inferior–superior dimension; jaw convex hull area was larger in speakers with slower speaking rates. Visual inspection of movement trajectories revealed that overall spatial kinematic characteristics in speakers with severe dysarthria differed qualitatively from those in speakers with mild or moderate dysarthria. Unlike speakers with dysarthria, typically aging speakers displayed variable tongue movement and minimal jaw movement. Conclusions The current study revealed that spatial articulatory characteristics, unlike temporal characteristics, showed a complicated pattern across the severity spectrum. The findings suggest that articulatory characteristics in speakers with severe dysarthria cannot simply be extrapolated from those in speakers with mild-to-moderate dysarthria secondary to ALS.

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Stem cell-derived cranial and spinal motor neurons reveal proteostatic differences between ALS resistant and sensitive motor neurons

eLife ◽

10.7554/elife.44423 ◽

2019 ◽

Vol 8 ◽

Cited By ~ 4

Author(s):

Disi An ◽

Ryosuke Fujiki ◽

Dylan E Iannitelli ◽

John W Smerdon ◽

Shuvadeep Maity ◽

...

Keyword(s):

Stem Cells ◽

Amyotrophic Lateral Sclerosis ◽

Motor Neurons ◽

Embryonic Stem ◽

Spinal Motor Neurons ◽

Spinal Motor ◽

Chemically Induced ◽

Late Stages ◽

Lateral Sclerosis ◽

Over Time

In amyotrophic lateral sclerosis (ALS) spinal motor neurons (SpMN) progressively degenerate while a subset of cranial motor neurons (CrMN) are spared until late stages of the disease. Using a rapid and efficient protocol to differentiate mouse embryonic stem cells (ESC) to SpMNs and CrMNs, we now report that ESC-derived CrMNs accumulate less human (h)SOD1 and insoluble p62 than SpMNs over time. ESC-derived CrMNs have higher proteasome activity to degrade misfolded proteins and are intrinsically more resistant to chemically-induced proteostatic stress than SpMNs. Chemical and genetic activation of the proteasome rescues SpMN sensitivity to proteostatic stress. In agreement, the hSOD1 G93A mouse model reveals that ALS-resistant CrMNs accumulate less insoluble hSOD1 and p62-containing inclusions than SpMNs. Primary-derived ALS-resistant CrMNs are also more resistant than SpMNs to proteostatic stress. Thus, an ESC-based platform has identified a superior capacity to maintain a healthy proteome as a possible mechanism to resist ALS-induced neurodegeneration.

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Quantitative Description of the Dysarthria in Women With Amyotrophic Lateral Sclerosis

Journal of Speech Language and Hearing Research ◽

10.1044/jshr.3504.723 ◽

1992 ◽

Vol 35 (4) ◽

pp. 723-733 ◽

Cited By ~ 60

Author(s):

Jane Finley Kent ◽

Raymond D. Kent ◽

John C. Rosenbek ◽

Gary Weismer ◽

Ruth Martin ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Speech Intelligibility ◽

Word Identification ◽

Signal To Noise Ratio ◽

Quantitative Description ◽

Initial Position ◽

Control Group ◽

Acoustic Measures ◽

Phonetic Features ◽

Lateral Sclerosis

Speech intelligibility and its phonetic and acoustic correlates were studied in a group of 10 women with amyotrophic lateral sclerosis (ALS). Intelligibility assessment with a word-identification test indicated that the most disrupted phonetic features pertained to velopharyngeal valving, lingual function for consonant contrasts of place and manner, and syllable shape. An acoustic signature analysis based on trajectories of the first and second formants in selected monosyllabic test words revealed that the mean slope of the second formant (F 2 ) was reduced compared with that of a normal geriatric control group. This F 2 slope reduction is interpreted to reflect loss of lingual motoneurons. Acoustic measures of phonatory function for sustained vowel prolongation demonstrated abnormalities in fundamental frequency, perturbations of frequency (jitter) and amplitude (shimmer), and signal-to-noise ratio. The data for women with ALS are compared with data for a normal geriatric control group of women and with data for a group of 25 men with ALS (Kent et al., 1990). Although the overall ranking of errors was similar for males and females with ALS, men were more likely to have impairments of voicing in syllable-initial position.

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Speech Deterioration in Amyotrophic Lateral Sclerosis

Journal of Speech Language and Hearing Research ◽

10.1044/jshr.3406.1269 ◽

1991 ◽

Vol 34 (6) ◽

pp. 1269-1275 ◽

Cited By ~ 42

Author(s):

Raymond D. Kent ◽

Robert L. Sufit ◽

John C. Rosenbek ◽

Jane F. Kent ◽

Gary Weismer ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Pulmonary Function ◽

Speech Intelligibility ◽

Word Identification ◽

Signal To Noise Ratio ◽

Observation Interval ◽

Formant Frequency ◽

Phonetic Features ◽

Second Formant ◽

Lateral Sclerosis

Few detailed reports have been published on the nature of speech and voice changes during the course of amyotrophic lateral sclerosis (ALS). The subject of this case study is a woman who was diagnosed as having ALS with bulbar signs at the age of 53. Speech intelligibility, pulmonary function, and selected speech and voice functions were tested during an approximately 2-year course of her disease. Over this period, her speech intelligibility, as measured by a multiplechoice word identification test, declined from 98% to 48%. Phonetic features that were most affected during the intelligibility decline included voicing contrast for syllable-initial and syllablefinal consonants, place of articulation contrasts for lingual consonants, manner of articulation for lingual consonants, stop versus nasal manner of production, features related to the liquid consonants, and various features related to syllable shape. An acoustic measure, average slope of the second-formant frequency, declined in association with the intelligibility reduction and is thought to reflect the loss of lingual motoneurons. Her pulmonary function also declined over the observation interval, with particularly severe reduction in measures of air flow. Oral diadochokinesis and measures of vocal function (including jitter, shimmer, and signal-to-noise ratio) were highly variable across test sessions. These results are discussed in terms of the challenges they present to sensitive assessment of change and to management of the communication disability in ALS.

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