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

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.

Author(s):  
Panying Rong ◽  
Lindsey Heidrick

Purpose This study examined the articulatory control of speech and speechlike tasks in individuals with amyotrophic lateral sclerosis (ALS) and neurologically healthy individuals with the aim to identify the most useful set of articulatory features and tasks for assessing bulbar motor involvement in ALS. Method Tongue and jaw kinematics were recorded in 12 individuals with bulbar ALS and 10 healthy controls during a speech task and two speechlike tasks (i.e., alternating motion rate [AMR], sequential motion rate [SMR]). Eight articulatory features were derived for each participant per task, including the range, maximum speed, and acceleration time of tongue and jaw movements as well as the coupling and timing between tongue and jaw movements. The effects of task (i.e., AMR, SMR, speech) and group (i.e., ALS, control) on these articulatory features were evaluated. For each feature, the task that yielded the largest difference between the ALS and control groups was identified. The diagnostic efficacy of these task-specific features was assessed using the receiver operating characteristic analysis; the relation of these task-specific features to a well-established bulbar severity index—speaking rate—was determined using Spearman's rank correlation. Results Seven task-specific articulatory features were identified, including (a) tongue and jaw acceleration time during the AMR task, (b) tongue–jaw coupling during the SMR task, and (c) range of tongue movement, maximum tongue and jaw speed, and temporal lag between tongue and jaw movements during the speech task. Among these features, tongue and jaw acceleration time and their temporal lag showed relatively high accuracy (i.e., 0.83–0.95) in differentiating individuals with ALS from healthy controls. Range of tongue movement and maximum tongue and jaw speed showed significant correlations with speaking rate. Conclusion Findings provided preliminary evidence for the utility of task-specific articulatory measurements as a novel quantitative assessment to detect and predict bulbar motor involvement in ALS.


2020 ◽  
Vol 63 (10) ◽  
pp. 3453-3460
Author(s):  
Michal Novotny ◽  
Jan Melechovsky ◽  
Kriss Rozenstoks ◽  
Tereza Tykalova ◽  
Petr Kryze ◽  
...  

Purpose The purpose of this research note is to provide a performance comparison of available algorithms for the automated evaluation of oral diadochokinesis using speech samples from patients with amyotrophic lateral sclerosis (ALS). Method Four different algorithms based on a wide range of signal processing approaches were tested on a sequential motion rate /pa/-/ta/-/ka/ syllable repetition paradigm collected from 18 patients with ALS and 18 age- and gender-matched healthy controls (HCs). Results The best temporal detection of syllable position for a 10-ms tolerance value was achieved for ALS patients using a traditional signal processing approach based on a combination of filtering in the spectrogram, Bayesian detection, and polynomial thresholding with an accuracy rate of 74.4%, and for HCs using a deep learning approach with an accuracy rate of 87.6%. Compared to HCs, a slow diadochokinetic rate ( p < .001) and diadochokinetic irregularity ( p < .01) were detected in ALS patients. Conclusions The approaches using deep learning or multiple-step combinations of advanced signal processing methods provided a more robust solution to the estimation of oral DDK variables than did simpler approaches based on the rough segmentation of the signal envelope. The automated acoustic assessment of oral diadochokinesis shows excellent potential for monitoring bulbar disease progression in individuals with ALS.


2018 ◽  
Vol 27 (1) ◽  
pp. 258-269 ◽  
Author(s):  
Jimin Lee ◽  
Michael Bell ◽  
Zachary Simmons

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.


2017 ◽  
Vol 60 (4) ◽  
pp. 810-825 ◽  
Author(s):  
Jeff Searl ◽  
Stephanie Knollhoff ◽  
Richard J. Barohn

Purpose This preliminary study on lingual–alveolar contact pressures (LACP) in people with amyotrophic lateral sclerosis (ALS) had several aims: (a) to evaluate whether the protocol induced fatigue, (b) to compare LACP during speech (LACP-Sp) and during maximum isometric pressing (LACP-Max) in people with ALS (PALS) versus healthy controls, (c) to compare the percentage of LACP-Max utilized during speech (%Max) for PALS versus controls, and (d) to evaluate relationships between LACP-Sp and LACP-Max with word intelligibility. Method Thirteen PALS and 12 healthy volunteers produced /t, d, s, z, l, n/ sounds while LACP-Sp was recorded. LACP-Max was obtained before and after the speech protocol. Word intelligibility was obtained from auditory–perceptual judgments. Results LACP-Max values measured before and after completion of the speech protocol did not differ. LACP-Sp and LACP-Max were statistically lower in the ALS bulbar group compared with controls and PALS with only spinal symptoms. There was no statistical difference between groups for %Max. LACP-Sp and LACP-Max were correlated with word intelligibility. Conclusions It was feasible to obtain LACP-Sp measures without inducing fatigue. Reductions in LACP-Sp and LACP-Max for bulbar speakers might reflect tongue weakness. Although confirmation of results is needed, the data indicate that individuals with high word intelligibility maintained LACP-Sp at or above 2 kPa and LACP-Max at or above 50 kPa.


2019 ◽  
Vol 2019 ◽  
pp. 1-11 ◽  
Author(s):  
Yana Yunusova ◽  
Jamal Ansari ◽  
Joel Ramirez ◽  
Sanjana Shellikeri ◽  
Greg J. Stanisz ◽  
...  

The goal of this study was to identify neurostructural frontal lobe correlates of cognitive and speaking rate changes in amyotrophic lateral sclerosis (ALS). 17 patients diagnosed with ALS and 12 matched controls underwent clinical, bulbar, and neuropsychological assessment and structural neuroimaging. Neuropsychological testing was performed via a novel computerized frontal battery (ALS-CFB), based on a validated theoretical model of frontal lobe functions, and focused on testing energization, executive function, emotion processing, theory of mind, and behavioral inhibition via antisaccades. The measure of speaking rate represented bulbar motor changes. Neuroanatomical assessment was performed using volumetric analyses focused on frontal lobe regions, postcentral gyrus, and occipital lobes as controls. Partial least square regressions (PLS) were used to predict behavioral (cognitive and speech rate) outcomes using volumetric measures. The data supported the overall hypothesis that distinct behavioral changes in cognition and speaking rate in ALS were related to specific regional neurostructural brain changes. These changes did not support a notion of a general dysexecutive syndrome in ALS. The observed specificity of behavior-brain changes can begin to provide a framework for subtyping of ALS. The data also support a more integrative framework for clinical assessment of frontal lobe functioning in ALS, which requires both behavioral testing and neuroimaging.


1988 ◽  
Vol 97 (2) ◽  
pp. 164-172 ◽  
Author(s):  
Lorraine A. Ramig ◽  
Ingo R. Titze ◽  
Ronald C. Scherer ◽  
Steven P. Ringel

This paper presents a rationale for acoustic analysis of voices of neurologically diseased patients, and reports preliminary data from patients with myotonic dystrophy, Huntington's disease, Parkinson's disease, and amyotrophic lateral sclerosis, as well as from individuals at risk for Huntington's disease. Noninvasive acoustic analysis may be of clinical value to the otolaryngologist, neurologist, and speech pathologist for early and differential diagnosis and for documenting disease progression in these various neurologic disorders.


2019 ◽  
Vol 90 (11) ◽  
pp. 1215-1220 ◽  
Author(s):  
Alexander G Thompson ◽  
Elizabeth Gray ◽  
Alexander Bampton ◽  
Dominika Raciborska ◽  
Kevin Talbot ◽  
...  

ObjectiveTo evaluate the classifier performance, clinical and biochemical correlations of cerebrospinal fluid (CSF) levels of the chitinase proteins Chitotriosidase-1 (CHIT1), Chitinase-3-like protein 1 (CHI3L1) and Chitinase-3-like protein 2 (CHI3L2) in amyotrophic lateral sclerosis (ALS).MethodsCSF levels of CHIT1, CHI3L1, CHI3L2, phosphorylated neurofilament heavy chain (pNFH) and C-reactive protein were measured by ELISA in a longitudinal cohort of patients with ALS (n=82), primary lateral sclerosis (PLS, n=10), ALS-mimic conditions (n=12), healthy controls (n=25) and asymptomatic carriers of ALS-causing genetic mutations (AGC; n=5).ResultsCSF CHIT1, CHI3L1 and CHI3L2 were elevated in patients with ALS compared with healthy controls (p<0.001) and ALS-mimics (CHIT1, p<0.001; CHI3L1, p=0.017; CHI3L2, p<0.001). CHIT1 and CHI3L2 were elevated in ALS compared with PLS (CHIT1, p=0.021; CHI3L1, p=0.417; CHI3L2, p<0.001). Chitinase levels were similar in AGCs and healthy controls. Chitinase proteins distinguished ALS from healthy controls (area under the curve (AUC): CHIT1 0.92; CHI3L1 0.80; CHI3L2 0.90), mimics (AUC: CHIT1 0.84; CHI3L1 0.73; CHI3L2 0.88) and, to a lesser extent, PLS (AUC: CHIT 0.73; CHI3L1 0.51; CHI3L2 0.82) but did not outperform pNFH. CHIT1 and CHI3L2 correlated with disease progression rate (Pearson’s r=0.49, p<0.001; r=0.42, p<0.001, respectively). CHI3L1 correlated with degree of cognitive dysfunction (r=−0.25, p=0.038). All chitinases correlated with pNFH. CHIT1 levels were associated with survival in multivariate models. Chitinase levels were longitudinally stable.ConclusionsCSF chitinase proteins may have limited value as independent diagnostic and stratification biomarkers in ALS, but offer a window into non-autonomous mechanisms of motor neuronal loss in ALS, specifically in assessing response to therapies targeting neuroinflammatory pathways.


2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Lu Chen ◽  
Yong Chen ◽  
Mingming Zhao ◽  
Lemin Zheng ◽  
Dongsheng Fan

Abstract To compare the plasma concentrations of trimethylamine N-oxide (TMAO) and its precursors in amyotrophic lateral sclerosis (ALS) patients, their spouses and healthy controls and to find associations between gut microbiota metabolites and ALS. ALS patients were recruited at Peking University Third Hospital from January 2015 to December 2018. Information was collected from their spouses at the same time. Age and gender matched healthy controls were recruited from individuals who visited the physical examination center for health checkups. Blood samples were collected after at least 4 h of fasting. Concentrations of the metabolites were quantified using stable isotope dilution liquid chromatography–tandem mass spectrometry. Group differences were analyzed using parametric and nonparametric tests, as appropriate. In this study, 160 patients with ALS were recruited. In these patients, 63 were compared with their spouses, 148 were compared with age and gender matched controls, and 60 were compared with both their spouses and heathy controls in the same time. The carnitine concentration was significantly higher in patients than in their spouses, while there were no significant differences in the concentrations of other metabolites. The carnitine and betaine concentrations were higher, while the choline, TMAO and butyrobetaine concentrations were lower in ALS than in healthy controls. The concentrations of the metabolites in the spouses were more similar to the ALS patients rather than to the healthy controls. In the ALS group, the plasma concentrations of carnitine, betaine, choline and TMAO were inversely related to the severity of upper motor neuron impairment. The TMAO metabolic pathway of the gut microbiota is disturbed in both ALS patients and their spouses, which might suggest that the changes in the gut microbiota occurred before disease onset. The negative correlations between the involvement of UMNs and the concentrations of the metabolites might suggest that the inhibition of this metabolic pathway might lead to a better prognosis in ALS patients.


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