Spectral Properties of Fricatives in Amyotrophic Lateral Sclerosis

1997 ◽  
Vol 40 (6) ◽  
pp. 1358-1372 ◽  
Author(s):  
Kris Tjaden ◽  
Greg S. Turner
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Spectral Characteristics ◽  
Measurement Techniques ◽  
Significant Proportion ◽  
Healthy Controls ◽  
Spectral Measures ◽  
First Moment ◽  
First Four Moments ◽  
The Relationship ◽  
Lateral Sclerosis

Spectral characteristics of word initial /s/and /∫/produced by individuals with amyotrophic lateral sclerosis (ALS) and healthy controls were quantified using spectral moment analysis. Coefficients of the first four moments were used as descriptive indices of central tendency, spread of energy, shape, and peakedness. The relationship between select moment coefficients and consonant precision ratings was investigated to explore potential perceptual correlates of the acoustic measures. To examine whether spectra for ALS and healthy speakers were influenced similarly by context, first moment coefficients for three occurrences of /s/in “some” were inspected for individual speakers. Select moment coefficients also were compared to hand-derived spectral measures for a subset of speakers. Results suggest articulatory differences in /s/and /∫/for the present group of ALS speakers and healthy controls, as indexed by between-group differences in first moment coefficients. A linear function accounted for a significant proportion of the variance in the relationship between consonant precision ratings and the frequency difference between the first moment for /s/and /∫/. Inspection of first moment coefficients for multiple occurrences of /s/in “some” suggests context influences the first moment in a similar manner for ALS and healthy speakers. The subanalysis comparing moment coefficients and hand-derived spectral measures revealed both similarities and differences in the manner the two measurement techniques reflect spectral shape.

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 Lingual–Alveolar Contact Pressure During Speech in Amyotrophic Lateral Sclerosis: Preliminary Findings

2017 ◽  
Vol 60 (4) ◽  
pp. 810-825 ◽  
Author(s):  
Jeff Searl ◽  
Stephanie Knollhoff ◽  
Richard J. Barohn
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Healthy Volunteers ◽  
Contact Pressure ◽  
Statistical Difference ◽  
Healthy Controls ◽  
Perceptual Judgments ◽  
Before And After ◽  
Preliminary Study ◽  
Contact Pressures ◽  
Lateral Sclerosis

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.

scholarly journals CSF chitinase proteins in amyotrophic lateral sclerosis

2019 ◽  
Vol 90 (11) ◽  
pp. 1215-1220 ◽  
Author(s):  
Alexander G Thompson ◽  
Elizabeth Gray ◽  
Alexander Bampton ◽  
Dominika Raciborska ◽  
Kevin Talbot ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Area Under The Curve ◽  
Neuronal Loss ◽  
Progression Rate ◽  
Healthy Controls ◽  
Reactive Protein ◽  
Classifier Performance ◽  
Disease Progression Rate ◽  
Csf Levels ◽  
Lateral Sclerosis

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.

scholarly journals Changes in the concentrations of trimethylamine N-oxide (TMAO) and its precursors in patients with amyotrophic lateral sclerosis

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Lu Chen ◽  
Yong Chen ◽  
Mingming Zhao ◽  
Lemin Zheng ◽  
Dongsheng Fan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Gut Microbiota ◽  
Metabolic Pathway ◽  
Plasma Concentrations ◽  
Disease Onset ◽  
Healthy Controls ◽  
Age And Gender ◽  
And Gender ◽  
Als Patients ◽  
Lateral Sclerosis

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.

Identification of GGC repeat expansion in the NOTCH2NLC gene in amyotrophic lateral sclerosis

Neurology ◽  
2020 ◽  
Vol 95 (24) ◽  
pp. e3394-e3405 ◽  
Author(s):  
Yanchun Yuan ◽  
Zhen Liu ◽  
Xuan Hou ◽  
Wanzhen Li ◽  
Jie Ni ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mainland China ◽  
Repeat Expansion ◽  
Fisher Exact Test ◽  
Healthy Controls ◽  
Exact Test ◽  
Repeat Expansions ◽  
Neuronal Intranuclear Inclusion ◽  
Electrophysiologic Studies ◽  
Lateral Sclerosis

ObjectiveTo determine whether the GGC repeats in the NOTCH2NLC gene contribute to amyotrophic lateral sclerosis (ALS).MethodsIn this study, 545 patients with ALS and 1,305 healthy controls from mainland China were recruited. Several pathogenic mutations in known ALS-causative genes (including C9ORF72 and ATXN2) and polynucleotide repeat expansions in NOP56 and AR genes were excluded. Repeat-primed PCR and GC-rich PCR were performed to determine the GGC repeat size in NOTCH2NLC. Systematic and targeted clinical evaluations and investigations, including skin biopsy and dynamic electrophysiologic studies, were conducted in the genetically affected patients.ResultsGGC repeat expansion was observed in 4 patients (numbers of repeats 44, 54, 96, and 143), accounting for ≈0.73% (4 of 545) of all patients with ALS. A comparison with 1,305 healthy controls revealed that GGC repeat expansion in NOTCH2NLC was associated with ALS (Fisher exact test, 4 of 545 vs 0 of 1,305, p = 0.007). Compared to patients with the neuronal intranuclear inclusion disease (NIID) muscle weakness–dominant subtype, patients with ALS phenotype carrying the abnormal repeat expansion tended to have a severe phenotype and rapid deterioration.ConclusionOur results suggest that ALS is a specific phenotype of NIID or that GGC expansion in NOTCH2NLC is a factor that modifies ALS. These findings may help clarify the pathogenic mechanism of ALS and may expand the known clinical spectrum of NIID.

Acoustic Differences Between Content and Function Words in Amyotrophic Lateral Sclerosis

2000 ◽  
Vol 43 (3) ◽  
pp. 769-781 ◽  
Author(s):  
Greg S. Turner ◽  
Kris Tjaden
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Vowel Duration ◽  
Function Words ◽  
Temporal Properties ◽  
Vowel Space ◽  
The Difference ◽  
And Function ◽  
Impaired Speech ◽  
The Relationship ◽  
Lateral Sclerosis

Studies describing acoustic characteristics of speech produced by individuals with dysarthria may help to explain intelligibility deficits for these speakers. One goal of the current study was to investigate the manner and extent to which nine speakers with mild to moderate dysarthria associated with amyotrophic lateral sclerosis (ALS) and nine healthy speakers acoustically distinguished /i/, / æ/, /u/, and /α/ in content and function words. A further aim was to evaluate the relationship between impaired speech in ALS and the magnitude of acoustic differences for vowels in content and function words. Speakers read the Farm Passage at a comfortable or habitual rate. F1 and F2 midpoint frequencies were measured, and vowel space areas were calculated. Vowel durations also were measured. The magnitude of F1, F2, vowel space area, and duration differences for vowels in content and function words was not statistically different for speakers with ALS and healthy controls. In addition, with the exception of /i/ produced by some speakers with ALS, vowel duration tended to be shorter in function words. Average F1 and F2 values for function words also tended to be centralized relative to content words. Although vowel space area differences for the two speaker groups were not statistically significant, there was a tendency for the difference in vowel space area for content and function words to be smaller for speakers with ALS than for controls. Regression analyses further indicated that the magnitude of temporal differences for vowels in content and function words was a better predictor of impaired speech than the magnitude of spectral differences for vowels in content and function words. One clinical implication is that individuals with ALS may benefit from therapy techniques targeting temporal properties of the acoustic signal.

The relationship between Bunina bodies, skein-like inclusions and neuronal loss in amyotrophic lateral sclerosis

2002 ◽  
Vol 103 (6) ◽  
pp. 583-589 ◽  
Author(s):  
M. van Welsem ◽  
Hogenhuis J. ◽  
Meininger V. ◽  
Metsaars W. ◽  
Hauw J.-J. ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Neuronal Loss ◽  
Bunina Bodies ◽  
The Relationship ◽  
Lateral Sclerosis

scholarly journals Poor Bone Quality in Patients With Amyotrophic Lateral Sclerosis

2020 ◽  
Vol 11 ◽  
Author(s):  
Jordi Caplliure-Llopis ◽  
Dolores Escrivá ◽  
María Benlloch ◽  
José Enrique de la Rubia Ortí ◽  
José María Estrela ◽  
...  
Keyword(s):  
Vitamin D ◽  
Amyotrophic Lateral Sclerosis ◽  
Bone Health ◽  
Clinical Status ◽  
Healthy Controls ◽  
Healthy Individuals ◽  
Bone Parameters ◽  
Als Patients ◽  
Lateral Sclerosis ◽  
Tsh Levels

Objective: Musculoskeletal functional deterioration in Amyotrophic lateral sclerosis (ALS) is associated with an increase in bone fractures. The purpose of this study was to evaluate the influence of sex, ALS type, on bone quality in patients with ALS compared to healthy controls. The impact on bone health of the clinical status and some metabolic parameters was also analyzed in ALS patients.Methods: A series of 33 voluntary patients with ALS, and 66 healthy individuals matched in sex and age underwent assessment of bone mass quality using quantitative ultrasound (QUS) of the calcaneus. Ultrasonic broadband attenuation (BUA), the speed of sound (SOS), stiffness index and T-score were measured. Bone mineral density (BMD) was estimated using standard equations. Apart from fat and muscle mass percentage determinations, clinical baseline measures in ALS patients included ALSFRS-R score, Barthel index for activities of daily living, pulmonary function measured using FVC, and muscular strength assessed by a modified MRC grading scale. Laboratory tests included serum calcium, 25-HO-cholecalciferol (Vitamin D), alkaline phosphatase (ALP), T4 and TSH.Results: All bone parameters evaluated were statistically significant lower in ALS patients than in healthy controls. ALS females showed significantly lower bone parameters than healthy females. According to the estimated BMD, there were 25 ALS patients (75.8%) and 36 (54.5%) healthy individuals showing an osteoporotic profile (BMD &lt;0.700 g/cm2). Only 16.7% of the ALS females had T-scores indicative of healthy bones. There was no correlation between any of the clinical parameters analyzed and the bone QUS measurements. Vitamin D and TSH levels positively correlated with all the bone parameters.Conclusions: This study confirms that ALS patients, particularly females, exhibited deteriorated bone health as compared to healthy individuals. These structural bone changes were independent of ALS subtype and clinical status. Bone health in ALS patients seems to be related to certain metabolic parameters such as Vitamin D and TSH levels.

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