Verbal expressive language minimally affected in non-demented people living with amyotrophic lateral sclerosis.

Objective: Language dysfunction is one of the most common cognitive impairments in amyotrophic lateral sclerosis (ALS). Although discourse capacities are essential for daily functioning, verbal expressive language has not been widely investigated in ALS. The existing research available suggests that discourse impairments are prevalent. This study investigates verbal expressive language in people living with ALS (plwALS) in contrast to healthy controls (HC).Methods: 64 plwALS and 49 age, gender and education-matched healthy controls were ask to describe the Cookie Theft Picture Task. The recordings were analyzed for discourse productivity, discourse content, syntactic complexity, speech fluency and verb processing. We applied the Bayesian hypothesis-testing framework, incorporating the effects of dysarthria, cognitive impairment status (CIS), and premorbid crystalline verbal IQ.Results: Compared to HC, plwALS only showed a single impairment: speech dysfluency. Discourse productivity, discourse content, syntactic complexity and verb processing were not impaired. Cognition and dysarthria exceeded the influence of verbal IQ for total words spoken and content density. Cognition alone seemed to explain dysfluency. Body-agent verbs were produced at even higher rates than other verb types. For the remaining outcomes, verbal IQ was the most decisive factor.Conclusions: In contrast to existing research, our data demonstrates no discernible impairment in verbal expressive language in ALS. What our findings show to be decisive is accounting for the influence of dysarthria, cognitive impairment status, and verbal IQ as variables on spontaneous verbal expressive language. Minor impairments in verbal expressive language appear to be influenced to a greater degree by executive dysfunctioning and dysarthria than by language impairment.

Behavioral Management of Respiratory/Phonatory Dysfunction for Dysarthria Associated With Neurodegenerative Disease: A Systematic Review.

PURPOSE: This systematic review represents an update to previous reviews of the literature addressing behavioral management of respiratory/phonatory dysfunction in individuals with dysarthria due to neurodegenerative disease. METHOD: Multiple electronic database searches and hand searches of prominent speech-language pathology journals were conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards. RESULTS: The search yielded 1,525 articles, from which 88 met inclusion criteria and were reviewed by two blinded co-investigators. A large range of therapeutic approaches have been added to the evidence base since the last review, including expiratory muscle strength training, singing, and computer- and device-driven programs, as well as a variety of treatment modalities, including teletherapy. Evidence for treatment in several different population groups-including cerebellar ataxia, myotonic dystrophy, autosomal recessive spastic ataxia of Charlevoix-Saguenay, Huntington's disease, multiple system atrophy, and Lewy body dementia-were added to the current review. Synthesis of evidence quality provided strong evidence in support of only one behavioral intervention: Lee Silverman Voice Treatment Program (LSVT LOUD) in people with Parkinson's disease. No other treatment approach or population included in this review demonstrated more than limited evidence, reflecting that these approaches/populations require urgent further examination. CONCLUSION: Suggestions about where future research efforts could be significantly strengthened and how clinicians can apply research findings to their practice are provided. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24964473.

Levodopa may modulate specific speech impairment in Parkinson's disease: an fMRI study.

Hypokinetic dysarthria (HD) is a difficult-to-treat symptom affecting quality of life in patients with Parkinson's disease (PD). Levodopa may partially alleviate some symptoms of HD in PD, but the neural correlates of these effects are not fully understood. The aim of our study was to identify neural mechanisms by which levodopa affects articulation and prosody in patients with PD. Altogether 20 PD patients participated in a task fMRI study (overt sentence reading). Using a single dose of levodopa after an overnight withdrawal of dopaminergic medication, levodopa-induced BOLD signal changes within the articulatory pathway (in regions of interest; ROIs) were studied. We also correlated levodopa-induced BOLD signal changes with the changes in acoustic parameters of speech. We observed no significant changes in acoustic parameters due to acute levodopa administration. After levodopa administration as compared to the OFF dopaminergic condition, patients showed task-induced BOLD signal decreases in the left ventral thalamus (p = 0.0033). The changes in thalamic activation were associated with changes in pitch variation (R = 0.67, p = 0.006), while the changes in caudate nucleus activation were related to changes in the second formant variability which evaluates precise articulation (R = 0.70, p = 0.003). The results are in line with the notion that levodopa does not have a major impact on HD in PD, but it may induce neural changes within the basal ganglia circuitries that are related to changes in speech prosody and articulation.

Sex Differences in the Speech of Persons With and Without Parkinson's Disease.

BACKGROUND: Sex differences are apparent in the prevalence and the clinical presentation of Parkinson's disease (PD), but their effects on speech have been less studied. METHOD: Speech acoustics of persons with (34 females and 34 males) and without (age- and sex-matched) PD were examined, assessing the effects of PD diagnosis and sex on ratings of dysarthria severity and acoustic measures of phonation (fundamental frequency standard deviation, smoothed cepstral peak prominence), speech rate (net syllables per second, percent pause ratio), and articulation (articulatory-acoustic vowel space, release burst precision). RESULTS: Most measures were affected by PD (dysarthria severity, fundamental frequency standard deviation) and sex (smoothed cepstral peak prominence, net syllables per second, percent pause ratio, articulatory-acoustic vowel space), but without interactions between them. Release burst precision was differentially affected by sex in PD. Relative to those without PD, persons with PD produced fewer plosives with a single burst: females more frequently produced multiple bursts, whereas males more frequently produced no burst at all. CONCLUSIONS: Most metrics did not indicate that speech production is differentially affected by sex in PD. Sex was, however, associated with disparate effects on release burst precision in PD, which deserves further study. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.24388666.

The Change of Vocal Tract Length in People with Parkinson's Disease.

Hypokinetic dysarthria is one of the early symptoms of Parkinson's disease (PD) and has been proposed for early detection and also for monitoring of the progression of the disease. PD reduces the control of vocal tract muscles such as the tongue and lips and, therefore the length of the active vocal tract is altered. However, the change in the vocal tract length due to the disease has not been investigated. The aim of this study was to determine the difference in the apparent vocal tract length (AVTL) between people with PD and age-matched control healthy people. The phoneme, /a/ from the UCI Parkinson's Disease Classification Dataset and the Italian Parkinson's Voice and Speech Dataset were used and AVTL was calculated based on the first four formants of the sustained phoneme (F1-F4). The results show a correlation between Parkinson's disease and an increase in vocal tract length. The most sensitive feature was the AVTL calculated using the first formants of sustained phonemes (F1). The other significant finding reported in this article is that the difference is significant and only appeared in the male participants. However, the size of the database is not sufficiently large to identify the possible confounding factors such as the severity and duration of the disease, medication, age, and comorbidity factors.Clinical relevance-The outcomes of this research have the potential to improve the identification of early Parkinsonian dysarthria and monitor PD progression.

A preliminary study on self-care telemonitoring of dysarthria in spinal muscular atrophy.

Spinal muscular atrophy (SMA) is a rare neuromuscular disease which may cause impairments in oro-facial musculature. Most of the individuals with SMA present bulbar signs such as flaccid dysarthria which mines their abilities to speak and, as consequence, their psychic balance. To support clinicians, recent work has demonstrated the feasibility of video-based techniques for assessing the oro-facial functions in patients with neurological disorders such as amyotrophic lateral sclerosis. However, no work has so far focused on automatic and quantitative monitoring of dysarthria in SMA. To overcome limitations this work's aim is to propose a cloud-based store-and-forward telemonitoring system for automatic and quantitative evaluation of oro-facial muscles in individuals with SMA. The system integrates a convolutional neural network (CNN) aimed at identifying the position of facial landmarks from video recordings acquired via a web application by an SMA patient.Clinical relevance- The proposed work is in the preliminary stage, but it represents the first step towards a better understanding of the bulbar-functions' evolution in patients with SMA.

Evaluation of orofacial force-related measures using a novel measuring device: explanation of associations with speech rate in dysarthria.

BACKGROUND: The aim of this study was to examine the potential associations between orofacial force-related measures and speech rate in matched groups of 23 adults with dysarthria, and 69 healthy adults. RESEARCH DESIGN AND METHODS: A novel piezoresistive sensor-based device was utilized to obtain the orofacial maximum forces (OMFs) and rate of force development (RFD) measures. The study computed alternating motion rates (AMRs), sequential motion rates (SMRs), and articulation rate (AR) for all participants. The analysis included between-group comparisons and correlation analyses. The study also examined the reliability of the OMFs and RFD measures. RESULTS: Individuals with dysarthria exhibited significantly slower speech rates (approximately 41.89% to 56.53% slower) compared to the control group. Except for a few exceptions in the jaw, the dysarthria group demonstrated significantly lower OMFs and RFD measures. The correlation analysis revealed that OMFs were weakly to moderately correlated (r = .488-.674) and RFD measures were very weak to moderately correlated (r = .047-.578) with speech rate measures. CONCLUSIONS: The findings suggest that reduced OMFs and RFD measures may contribute to the slowed speech rate observed in adults with dysarthria. The study also highlights that OMFs are significantly more reliable (day-to-day) than RFD measures.

Improving the Efficiency of Dysarthria Voice Conversion System Based on Data Augmentation.

Dysarthria, a speech disorder often caused by neurological damage, compromises the control of vocal muscles in patients, making their speech unclear and communication troublesome. Recently, voice-driven methods have been proposed to improve the speech intelligibility of patients with dysarthria. However, most methods require a significant representation of both the patient's and target speaker's corpus, which is problematic. This study aims to propose a data augmentation-based voice conversion (VC) system to reduce the recording burden on the speaker. We propose dysarthria voice conversion 3.1 (DVC 3.1) based on a data augmentation approach, including text-to-speech and StarGAN-VC architecture, to synthesize a large target and patient-like corpus to lower the burden of recording. An objective evaluation metric of the Google automatic speech recognition (Google ASR) system and a listening test were used to demonstrate the speech intelligibility benefits of DVC 3.1 under free-talk conditions. The DVC system without data augmentation (DVC 3.0) was used for comparison. Subjective and objective evaluation based on the experimental results indicated that the proposed DVC 3.1 system enhanced the Google ASR of two dysarthria patients by approximately [62.4%, 43.3%] and [55.9%, 57.3%] compared to unprocessed dysarthria speech and the DVC 3.0 system, respectively. Further, the proposed DVC 3.1 increased the speech intelligibility of two dysarthria patients by approximately [54.2%, 22.3%] and [63.4%, 70.1%] compared to unprocessed dysarthria speech and the DVC 3.0 system, respectively. The proposed DVC 3.1 system offers significant potential to improve the speech intelligibility performance of patients with dysarthria and enhance verbal communication quality.

Subclinical articulatory changes of vowel parameters in Korean amyotrophic lateral sclerosis patients with perceptually normal voices.

The available quantitative methods for evaluating bulbar dysfunction in patients with amyotrophic lateral sclerosis (ALS) are limited. We aimed to characterize vowel properties in Korean ALS patients, investigate associations between vowel parameters and clinical features of ALS, and analyze subclinical articulatory changes of vowel parameters in those with perceptually normal voices. Forty-three patients with ALS (27 with dysarthria and 16 without dysarthria) and 20 healthy controls were prospectively collected in the study. Dysarthria was assessed using the ALS Functional Rating Scale-Revised (ALSFRS-R) speech subscores, with any loss of 4 points indicating the presence of dysarthria. The structured speech samples were recorded and analyzed using Praat software. For three corner vowels (/a/, /i/, and /u/), data on the vowel duration, fundamental frequency, frequencies of the first two formants (F1 and F2), harmonics-to-noise ratio, vowel space area (VSA), and vowel articulation index (VAI) were extracted from the speech samples. Corner vowel durations were significantly longer in ALS patients with dysarthria than in healthy controls. The F1 frequency of /a/, F2 frequencies of /i/ and /u/, the VSA, and the VAI showed significant differences between ALS patients with dysarthria and healthy controls. The area under the curve (AUC) was 0.912. The F1 frequency of /a/ and the VSA were the major determinants for differentiating ALS patients who had not yet developed apparent dysarthria from healthy controls (AUC 0.887). In linear regression analyses, as the ALSFRS-R speech subscore decreased, both the VSA and VAI were reduced. In contrast, vowel durations were found to be rather prolonged. The analyses of vowel parameters provided a useful metric correlated with disease severity for detecting subclinical bulbar dysfunction in ALS patients.

Optimising verbal fluency analysis in neurological patients with dysarthria: examples from Parkinson's disease and hereditary ataxia.

BACKGROUND: Verbal fluency tests (VFTs) are widely used to assess cognitive-linguistic performance in neurological diseases. However, the influence of dysarthria on performance in tests requiring oral responses is unclear in ataxia and Parkinson's disease. OBJECTIVES: To determine the impact of dysarthria on VFT performance and evaluate the validity and reliability of alternative methods for analyzing VFT data. METHOD: Trained raters evaluated dysarthria using VFT recordings in people with ataxia (N = 61) or Parkinson's disease (PD; N = 69). Total Correct Items scores and qualitative parameters (intrusions, ambiguous verbalizations, perseverations, and interjections) were compared across semantic, phonemic, and alternating fluency tasks. Disease severity was considered as a covariate in the regression model. RESULTS: VFT dysarthria ratings correlated with the benchmark (ground truth) dysarthria scores derived from a monologue. Ambiguous responses resulting from unclear speech impeded the rater's ability to determine if a response was correct. Regression analysis indicated that more severe dysarthria ratings predicted diminished scores in all three tasks (semantic fluency, phonemic fluency and alternating fluency) in the ataxia group. The contribution of disease severity to semantic, phonemic and alternating fluency was reduced substantially in the ataxia group after accounting for dysarthria severity in the model in both groups. CONCLUSIONS: Dysarthria severity can be estimated based on speech samples derived from VFT. Dysarthria can lead to lower total correct items and is associated with more ambiguous verbalizations in VFT. Dysarthria severity should be considered when interpreting VFT performance in common movement disorders.

Utilizing speech analysis to differentiate progressive supranuclear palsy from Parkinson's disease.

INTRODUCTION: Distinguishing Parkinson's disease (PD) from Progressive supranuclear palsy (PSP) at early disease stages is important for clinical trial enrollment and clinical care/prognostication. METHODS: We recruited 21 participants with PSP(n = 11) or PD(n = 10) with reliable caregivers. Standardized passage reading, counting, and sustained phonation were recorded on the BioDigit Home tablet (BioSensics LLC, Newton, MA USA), and speech features from the assessments were analyzed using the BioDigit Speech platform (BioSensics LLC, Newton, MA USA). An independent t-test was performed to compare each speech feature between PSP and PD participants. We also performed Spearman's correlations to evaluate associations between speech measures and clinical scores (e.g., PSP rating scales and MoCA). In addition, the model's performance in classifying PSP and PD was evaluated using Rainbow passage reading analysis. RESULTS: During Rainbow passage reading, PSP participants had a significantly slower articulation rate (2.45(0.49) vs 3.60(0.47) words/minute), lower speech-to-pause ratio (2.33(1.08) vs 3.67(1.18)), intelligibility dynamic time warping (DTW, 0.26(0.19) vs 0.53(0.26)), and similarity DTW (0.43(0.27) vs 0.67(0.13)) compared to PD participants. PSP participants also had longer pause times (17.24(5.47) vs 8.45(3.13) sec) and longer total signal times (52.44(6.67) vs (36.67(6.73) sec) when reading the passage. In terms of the phonation 'a', PSP participants showed a significant higher spectral entropy, spectral centroid, and spectral spread compared to PD participants and no differences were found for phonation 'e'. PD participants had more accurate reverse number counts than PSP participants (14.89(3.86) vs 7.36(4.67)). PSP Rating Scale (PSPRS) dysarthria (r = 0.79, p = 0.004) and bulbar item scores (r = 0.803, p = 0.005) were positively correlated with articulation rate in reverse number counts. Correct reverse number counts were positively correlated with total Montreal Cognitive Assessment scores (r = 0.703, p = 0.016). Machine learning models using passage reading-derived measures obtained an AUC of 0.93, and the sensitivity/specificity in correctly classifying PSP and PD participants were 0.95 and 0.90, respectively. CONCLUSION: Our study demonstrates the feasibility of differentiating PSP from PD using a digital health technology platform. Further multi-center studies are needed to expand and validate our initial findings.

Perceptual Learning of Dysarthria in Adolescence.

PURPOSE: As evidenced by perceptual learning studies involving adult listeners and speakers with dysarthria, adaptation to dysarthric speech is driven by signal predictability (speaker property) and a flexible speech perception system (listener property). Here, we extend adaptation investigations to adolescent populations and examine whether adult and adolescent listeners can learn to better understand an adolescent speaker with dysarthria. METHOD: Classified by developmental stage, adult (n = 42) and adolescent (n = 40) listeners completed a three-phase perceptual learning protocol (pretest, familiarization, and posttest). During pretest and posttest, all listeners transcribed speech produced by a 13-year-old adolescent with spastic dysarthria associated with cerebral palsy. During familiarization, half of the adult and adolescent listeners engaged in structured familiarization (audio and lexical feedback) with the speech of the adolescent speaker with dysarthria; and the other half, with the speech of a neurotypical adolescent speaker (control). RESULTS: Intelligibility scores increased from pretest to posttest for all listeners. However, listeners who received dysarthria familiarization achieved greater intelligibility improvements than those who received control familiarization. Furthermore, there was a significant effect of developmental stage, where the adults achieved greater intelligibility improvements relative to the adolescents. CONCLUSIONS: This study provides the first tranche of evidence that adolescent dysarthric speech is learnable-a finding that holds even for adolescent listeners whose speech perception systems are not yet fully developed. Given the formative role that social interactions play during adolescence, these findings of improved intelligibility afford important clinical implications.

The Influence of Sensorineural Hearing Loss on the Relationship Between the Perception of Speech in Noise and Dysarthric Speech.

PURPOSE: The ability to understand speech under adverse listening conditions is highly variable across listeners. Despite this, studies have found that listeners with normal hearing display consistency in their ability to perceive speech across different types of degraded speech, suggesting that, for at least these listeners, global skills may be involved in navigating the ambiguity in speech signals. However, there are substantial differences in the perceptual challenges faced by listeners with normal and impaired hearing. This study examines whether listeners with sensorineural hearing loss demonstrate the same type of consistency as normal-hearing listeners when processing neurotypical (i.e., control) speech that has been degraded by external noise and speech that is neurologically degraded such as dysarthria. METHOD: Listeners with normal hearing (n = 31) and listeners with sensorineural hearing loss (n = 36) completed an intelligibility task with neurotypical speech in noise and with dysarthric speech in quiet. RESULTS: Findings were consistent with previous work demonstrating a relationship between the ability to perceive neurotypical speech in noise and dysarthric speech for listeners with normal hearing, albeit at a higher intelligibility level than previously observed. This relationship was also observed for listeners with hearing loss, although listeners with more severe hearing losses performed better with dysarthric speech than with neurotypical speech in noise. CONCLUSIONS: This study demonstrated a high level of consistency in intelligibility performance for listeners across two different types of degraded speech, even when those listeners were further challenged by the presence of sensorineural hearing loss. Clinical implications for both listeners with hearing loss and their communication partners with dysarthria are discussed.

Global Acoustic Speech Temporal Characteristics for Mandarin Speakers With Parkinson's Disease During Syllable Repetition and Passage Reading.

PURPOSE: Previous research has revealed considerable variation in speech rates among English speakers with Parkinson's disease (PD) with slower, faster, or similar rates than controls. The purpose of this study was to characterize speech rates of Mandarin speakers with PD and the corresponding articulation and pause characteristics explaining the speech rates to enhance rate control therapies. METHOD: Eighteen Mandarin speakers with PD and 18 controls produced syllable repetitions and passage reading using their typical speech style. Speech rates, articulation rates, mean pause durations (≥ 10 ms), and the number of pauses with duration between 10 ms and 300 ms and greater than 300 ms were measured and compared between groups and tasks as well as across the initial, middle, and final periods of the passage. Two-way, mixed-model analyses of variance were employed for data analysis. RESULTS: Compared to controls, individuals with PD spoke with similar speech rates and faster articulation rates during passage reading, whereas during syllable repetitions, they produced slower speech rates and comparable articulation rates. The slower syllable repetitions produced by speakers with PD may be explained by longer pauses and more perceptual pauses, whereas faster articulation rates may explain the trend of faster speech rates during reading. Speech and articulation rates accelerated for both groups during passage reading. CONCLUSIONS: Speech rates of Mandarin speakers with PD were characterized by faster articulation, longer pauses, and more perceptual pauses for passage reading. A descriptive model of speech rate suggested that speakers with PD and dysarthria in this study would benefit from rate reduction therapy decreasing articulation rate. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.23982282.

Wilson Disease: A Case Report of Psychosis Preceding Parkinsonism.

BACKGROUND A first psychotic episode requires the exclusion of toxic-metabolic, inflammatory, infective, and neoplastic causes. Wilson disease is a rare, autosomal recessive disorder of copper metabolism and can present with neuropsychiatric symptoms secondary to copper accumulation in the brain. CASE REPORT We describe the case of a 48-year-old man with parkinsonism on a background of longstanding schizophrenia and psychotic depression in the setting of previously undiagnosed Wilson disease. The common history of neuropsychiatric disturbance and neuroleptic use complicated the assessment of parkinsonism. However, close attention to the temporal appearance of symptoms and signs differentiated his case from drug-induced parkinsonism, which commonly develops hours to weeks after commencement or uptitration of antipsychotic medication. The early features of sialorrhea and dysarthria were also atypical for idiopathic Parkinson disease. The diagnosis was confirmed by serum copper testing and supported by Kayser-Fleischer rings on bedside ophthalmological examination. Magnetic resonance imaging (MRI) of the brain demonstrated copper accumulation in the basal ganglia and pons, contributing to the characteristic neurological manifestations of an akinetic-rigid syndrome with dysarthria. CONCLUSIONS Serum copper testing is easily obtained and should be considered as part of the first-line investigations for new neuropsychiatric disturbances. Although rare, Wilson disease, if diagnosed early, is a potentially treatable and reversible cause of psychosis. With advanced disease, extrapyramidal findings on examination correlate with MRI brain changes, aiding the clinical assessment in differentiating the disease from drug-induced parkinsonism.

Extracting Phonetic Posterior-Based Features for Detecting Multiple Sclerosis From Speech.

Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system which, in addition to affecting motor and cognitive functions, may also lead to specific changes in the speech of patients. Speech production, comprehension, repetition and naming tasks, as well as structural and content changes in narratives, might indicate a limitation of executive functions. In this study we present a speech-based machine learning technique to distinguish speakers with relapsing-remitting subtype MS and healthy controls (HC). We exploit the fact that MS might cause a motor speech disorder similar to dysarthria, which, with our hypothesis, might affect the phonetic posterior estimates supplied by a Deep Neural Network acoustic model. From our experimental results, the proposed posterior posteriorgram-based feature extraction approach is useful for detecting MS: depending on the actual speech task, we obtained Equal Error Rate values as low as 13.3%, and AUC scores up to 0.891, indicating a competitive and more consistent classification performance compared to both the x-vector and the openSMILE 'ComParE functionals' attributes. Besides this discrimination performance, the interpretable nature of the phonetic posterior features might also make our method suitable for automatic MS screening or monitoring the progression of the disease. Furthermore, by examining which specific phonetic groups are the most useful for this feature extraction process, the potential utility of the proposed phonetic features could also be utilized in the speech therapy of MS patients.