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

2015 ◽  
Vol 2015 ◽  
pp. 1-11 ◽  
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.

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

2018 ◽  
Vol 27 (1) ◽  
pp. 258-269 ◽  
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.

scholarly journals Shorter Sentence Length Maximizes Intelligibility and Speech Motor Performance in Persons With Dysarthria Due to Amyotrophic Lateral Sclerosis

2019 ◽  
Vol 28 (1) ◽  
pp. 96-107 ◽  
Author(s):  
Kristen M. Allison ◽  
Yana Yunusova ◽  
Jordan R. Green
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Speech Intelligibility ◽  
Motor Performance ◽  
Pause Duration ◽  
Sentence Length ◽  
Speaking Rate ◽  
Event Duration ◽  
Severity Levels ◽  
Speech Motor ◽  
Lateral Sclerosis

Purpose The purpose of this study was to investigate the effect of sentence length on intelligibility and measures of speech motor performance in persons with amyotrophic lateral sclerosis (ALS) and to determine how these effects were influenced by dysarthria severity levels. Method One hundred thirty-one persons with ALS were included in this study, stratified into 4 dysarthria severity groups. All participants produced sentences from 5 to 15 words in length. Intelligibility, speaking rate, and measures of speech pausing behavior (i.e., total speech duration, total pause duration, and mean speech event duration) were measured for each sentence. Linear mixed-effects models were used to determine the effect of sentence length on speech measures for speakers at different dysarthria severity levels. Results Results showed that speech intelligibility significantly declined at longer sentence lengths only for the speakers with ALS who had more advanced dysarthria symptoms; however, speakers with mild-to-severe dysarthria showed significant declines in speaking rate and speech pausing behavior at longer sentence lengths. Conclusions Findings suggest that producing shorter sentences may help maximize intelligibility for speakers with moderate-to-severe dysarthria secondary to ALS and may be a beneficial compensatory strategy for preserving motor effort for all speakers with dysarthria secondary to ALS.

The Influence of Speaking Rate on Vowel Space and Speech Intelligibility for Individuals With Amyotrophic Lateral Sclerosis

1995 ◽  
Vol 38 (5) ◽  
pp. 1001-1013 ◽  
Author(s):  
Greg S. Turner ◽  
Kris Tjaden ◽  
Gary Weismer
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Speech Intelligibility ◽  
Speaking Rate ◽  
Age And Gender ◽  
Vowel Space ◽  
Global Estimates ◽  
And Gender ◽  
The Relationship ◽  
Neurologically Intact ◽  
Lateral Sclerosis

The relationship between speaking rate, vowel space area, and speech intelligibility was studied in a group of 9 subjects with amyotrophic lateral sclerosis (ALS) and 9 age- and gender-matched controls. Subjects read a standard passage (the Farm Passage) at three speaking rates, including HABITUAL, FAST, and SLOW. Vowel segment durations and target formant frequencies were measured at each speaking rate from select words containing the vowels /i/, /æ/, /a/, and /u/. To quantify changes in vowel space area across speaking rate, the area of the vowel quadrilateral was calculated for each speaker at each speaking rate. In addition, intelligibility estimates at each speaking rate were obtained for the dysarthric speakers. Results revealed that dysarthric speakers exhibited smaller vowel space areas and less systematic changes in vowel space as a function of speaking rate, when compared to the neurologically intact speakers. In an examination of the relationship between vowel space area and speech intelligibility, vowel space was found to account for 45% of the variance in speech intelligibility. This result suggests that vowel space area is an important component of global estimates of speech intelligibility.

Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 63 (1) ◽  
pp. 59-73 ◽  
Author(s):  
Panying Rong
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Acoustic Analysis ◽  
Speaking Rate ◽  
Disease Stage ◽  
Healthy Controls ◽  
Tongue Movement ◽  
Major Barrier ◽  
Syllable Repetition ◽  
Oral Diadochokinesis ◽  
Lateral Sclerosis

Purpose The purpose of this article was to validate a novel acoustic analysis of oral diadochokinesis (DDK) in assessing bulbar motor involvement in amyotrophic lateral sclerosis (ALS). Method An automated acoustic DDK analysis was developed, which filtered out the voice features and extracted the envelope of the acoustic waveform reflecting the temporal pattern of syllable repetitions during an oral DDK task (i.e., repetitions of /tɑ/ at the maximum rate on 1 breath). Cycle-to-cycle temporal variability (cTV) of envelope fluctuations and syllable repetition rate (sylRate) were derived from the envelope and validated against 2 kinematic measures, which are tongue movement jitter (movJitter) and alternating tongue movement rate (AMR) during the DDK task, in 16 individuals with bulbar ALS and 18 healthy controls. After the validation, cTV, sylRate, movJitter, and AMR, along with an established clinical speech measure, that is, speaking rate (SR), were compared in their ability to (a) differentiate individuals with ALS from healthy controls and (b) detect early-stage bulbar declines in ALS. Results cTV and sylRate were significantly correlated with movJitter and AMR, respectively, across individuals with ALS and healthy controls, confirming the validity of the acoustic DDK analysis in extracting the temporal DDK pattern. Among all the acoustic and kinematic DDK measures, cTV showed the highest diagnostic accuracy (i.e., 0.87) with 80% sensitivity and 94% specificity in differentiating individuals with ALS from healthy controls, which outperformed the SR measure. Moreover, cTV showed a large increase during the early disease stage, which preceded the decline of SR. Conclusions This study provided preliminary validation of a novel automated acoustic DDK analysis in extracting a useful measure, namely, cTV, for early detection of bulbar ALS. This analysis overcame a major barrier in the existing acoustic DDK analysis, which is continuous voicing between syllables that interferes with syllable structures. This approach has potential clinical applications as a novel bulbar assessment.

scholarly journals Improved Long-Term Survival with Edaravone Therapy in Patients with Amyotrophic Lateral Sclerosis: A Retrospective Single-Center Study in Japan

2021 ◽  
Vol 14 (8) ◽  
pp. 705
Author(s):  
Hideki Houzen ◽  
Takahiro Kano ◽  
Kazuhiro Horiuchi ◽  
Masahiro Wakita ◽  
Azusa Nagai ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Rank Test ◽  
Positive Pressure ◽  
Control Group ◽  
Single Center ◽  
Term Survival ◽  
Long Term Survival ◽  
Kaplan Meier ◽  
Lateral Sclerosis

Reports on the long-term survival effect of edaravone, which was approved for the treatment of amyotrophic lateral sclerosis (ALS) in 2015 in Japan, are rare. Herein, we report our retrospective analysis of 45 consecutive patients with ALS who initially visited our hospital between 2013 and 2018. Of these, 22 patients were treated with edaravone for an average duration of 26.6 (range, 2–64) months, whereas the remaining patients were not treated with edaravone and comprised the control group. There were no differences in baseline demographics between the two groups. The primary endpoint was tracheostomy positive-pressure ventilation (TPPV) or death, and the follow-up period ended in December 2020. The survival rate was significantly better in the edaravone group than in the control group based on the Kaplan–Meier analysis, which revealed that the median survival durations were 49 (9–88) and 25 (8–41) months in the edaravone and control groups, respectively (p = 0.001, log-rank test). There were no serious edaravone-associated adverse effects during the study period. Overall, the findings of this single-center retrospective study suggest that edaravone might prolong survival in patients with ALS.

scholarly journals The Skeletal Muscle Emerges as a New Disease Target in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 11 (7) ◽  
pp. 671
Author(s):  
Oihane Pikatza-Menoio ◽  
Amaia Elicegui ◽  
Xabier Bengoetxea ◽  
Neia Naldaiz-Gastesi ◽  
Adolfo López de Munain ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Amyotrophic Lateral Sclerosis ◽  
Muscle Tissue ◽  
Motor Neurons ◽  
Neurodegenerative Disorder ◽  
Disease Onset ◽  
Fine Tuning ◽  
Current State ◽  
The Central Nervous System ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder that leads to progressive degeneration of motor neurons (MNs) and severe muscle atrophy without effective treatment. Most research on ALS has been focused on the study of MNs and supporting cells of the central nervous system. Strikingly, the recent observations of pathological changes in muscle occurring before disease onset and independent from MN degeneration have bolstered the interest for the study of muscle tissue as a potential target for delivery of therapies for ALS. Skeletal muscle has just been described as a tissue with an important secretory function that is toxic to MNs in the context of ALS. Moreover, a fine-tuning balance between biosynthetic and atrophic pathways is necessary to induce myogenesis for muscle tissue repair. Compromising this response due to primary metabolic abnormalities in the muscle could trigger defective muscle regeneration and neuromuscular junction restoration, with deleterious consequences for MNs and thereby hastening the development of ALS. However, it remains puzzling how backward signaling from the muscle could impinge on MN death. This review provides a comprehensive analysis on the current state-of-the-art of the role of the skeletal muscle in ALS, highlighting its contribution to the neurodegeneration in ALS through backward-signaling processes as a newly uncovered mechanism for a peripheral etiopathogenesis of the disease.

scholarly journals LanCL1 promotes motor neuron survival and extends the lifespan of amyotrophic lateral sclerosis mice

2019 ◽  
Vol 27 (4) ◽  
pp. 1369-1382 ◽  
Author(s):  
Honglin Tan ◽  
Mina Chen ◽  
Dejiang Pang ◽  
Xiaoqiang Xia ◽  
Chongyangzi Du ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Motor Neurons ◽  
Neuronal Survival ◽  
Disease Onset ◽  
Alternative Mechanism ◽  
Specific Expression ◽  
Motor Neuron Survival ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive loss of motor neurons. Improving neuronal survival in ALS remains a significant challenge. Previously, we identified Lanthionine synthetase C-like protein 1 (LanCL1) as a neuronal antioxidant defense gene, the genetic deletion of which causes apoptotic neurodegeneration in the brain. Here, we report in vivo data using the transgenic SOD1G93A mouse model of ALS indicating that CNS-specific expression of LanCL1 transgene extends lifespan, delays disease onset, decelerates symptomatic progression, and improves motor performance of SOD1G93A mice. Conversely, CNS-specific deletion of LanCL1 leads to neurodegenerative phenotypes, including motor neuron loss, neuroinflammation, and oxidative damage. Analysis reveals that LanCL1 is a positive regulator of AKT activity, and LanCL1 overexpression restores the impaired AKT activity in ALS model mice. These findings indicate that LanCL1 regulates neuronal survival through an alternative mechanism, and suggest a new therapeutic target in ALS.

scholarly journals Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers

Brain ◽  
2014 ◽  
Vol 137 (11) ◽  
pp. 2938-2950 ◽  
Author(s):  
Axel Freischmidt ◽  
Kathrin Müller ◽  
Lisa Zondler ◽  
Patrick Weydt ◽  
Alexander E. Volk ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Expression Profiles ◽  
Consensus Sequence ◽  
Disease Onset ◽  
Therapeutic Targets ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Sequence Motif ◽  
Mutation Carriers ◽  
Disease Modifying ◽  
Lateral Sclerosis

Abstract Knowledge about the nature of pathomolecular alterations preceding onset of symptoms in amyotrophic lateral sclerosis is largely lacking. It could not only pave the way for the discovery of valuable therapeutic targets but might also govern future concepts of pre-manifest disease modifying treatments. MicroRNAs are central regulators of transcriptome plasticity and participate in pathogenic cascades and/or mirror cellular adaptation to insults. We obtained comprehensive expression profiles of microRNAs in the serum of patients with familial amyotrophic lateral sclerosis, asymptomatic mutation carriers and healthy control subjects. We observed a strikingly homogenous microRNA profile in patients with familial amyotrophic lateral sclerosis that was largely independent from the underlying disease gene. Moreover, we identified 24 significantly downregulated microRNAs in pre-manifest amyotrophic lateral sclerosis mutation carriers up to two decades or more before the estimated time window of disease onset; 91.7% of the downregulated microRNAs in mutation carriers overlapped with the patients with familial amyotrophic lateral sclerosis. Bioinformatic analysis revealed a consensus sequence motif present in the vast majority of downregulated microRNAs identified in this study. Our data thus suggest specific common denominators regarding molecular pathogenesis of different amyotrophic lateral sclerosis genes. We describe the earliest pathomolecular alterations in amyotrophic lateral sclerosis mutation carriers known to date, which provide a basis for the discovery of novel therapeutic targets and strongly argue for studies evaluating presymptomatic disease-modifying treatment in amyotrophic lateral sclerosis.

scholarly journals A Phonetic Complexity-Based Approach for Intelligibility and Articulatory Precision Testing: A Preliminary Study on Talkers With Amyotrophic Lateral Sclerosis

2018 ◽  
Vol 61 (9) ◽  
pp. 2205-2214 ◽  
Author(s):  
Mili Kuruvilla-Dugdale ◽  
Claire Custer ◽  
Lindsey Heidrick ◽  
Richard Barohn ◽  
Raghav Govindarajan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Speech Intelligibility ◽  
Speech Impairments ◽  
Word Level ◽  
Preliminary Study ◽  
Perceived Impact ◽  
Complex Words ◽  
Level Analysis ◽  
Precision Testing ◽  
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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