Quantitative Description of the Dysarthria in Women With Amyotrophic Lateral Sclerosis

1992 ◽  
Vol 35 (4) ◽  
pp. 723-733 ◽  
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.

Speech Deterioration in Amyotrophic Lateral Sclerosis

1991 ◽  
Vol 34 (6) ◽  
pp. 1269-1275 ◽  
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.

Impairment of Speech Intelligibility in Men with Amyotrophic Lateral Sclerosis

1990 ◽  
Vol 55 (4) ◽  
pp. 721-728 ◽  
Author(s):  
Raymond D. Kent ◽  
Jane F. Kent ◽  
Gary Weismer ◽  
Robert L. Sufit ◽  
John C. Rosenbek ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Speech Intelligibility ◽  
Percent Correct ◽  
Error Proportion ◽  
Male Patients ◽  
Phonetic Features ◽  
Muscle Impairment ◽  
Intelligibility Score ◽  
Manner Of Articulation ◽  
Lateral Sclerosis

Speech intelligibility was studied in a group of 25 male patients with amyotrophic lateral sclerosis (ALS). The object of the study was to determine the phonetic impairments underlying the speech intelligibility deficits that frequently accompany ALS. Analyses with a word intelligibility test indicated that the most disrupted phonetic features involved phonatory (voicing contrast) function, velopharyngeal valving, place and manner of articulation for lingual consonants, and regulation of tongue height for vowels. The mean error proportion for the five most severely affected features correlated highly (0.97) with the intelligibility score (percent correct). The phonetic feature analyses are one index of bulbar muscle impairment in amyotrophic lateral sclerosis and also may help to direct the speech management in these individuals.

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 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.

scholarly journals Significance of Serological Markers in Neurological Function and Progression Rate of Amyotrophic Lateral Sclerosis

2021 ◽  
Author(s):  
Xiaowen Chen ◽  
Junrong Li ◽  
Yingying Lv ◽  
Wei Zhang ◽  
xiujian Xu ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Creatine Kinase ◽  
Uric Acid ◽  
Total Cholesterol ◽  
Cystatin C ◽  
Control Group ◽  
Progression Rate ◽  
Cholesterol Levels ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Objective The present study was to investigate the significance of creatinine, uric acid, creatine kinase, total cholesterol, triglyceride, HCY (Homocysteine), and cystatin C in neurological function and progression rate of amyotrophic lateral sclerosis. Methods According to the diagnostic criteria of EI-Escorial (2000), 103 patients with ALS were enrolled. All patients were given corresponding serological tests at the initial diagnosis. The Revised ALS Functional Rating Scale (ALSFRS-R) and Disease Progression Rate (DPR) were evaluated. The detected indexes in blood tests included creatinine, uric acid, creatine kinase, total cholesterol, triglycerides, homocysteine, and cystatin C. All data were input into the computer, and the data analysis was performed by SPSS22.0 statistical software.Results 1. There were significant differences in creatinine, uric acid, creatine kinase,
total cholesterol, HCY and cystatin C between the two groups (P<0.05). The levels of uric acid and creatinine of ALS group were lower than those of the control group, and the levels of creatine kinase, total cholesterol, triglyceride, HCY and cystatin C were higher than that in the control group.
2. The results from correlation analysis demonstrated that there was a significant correlation between ALSFRS-R and creatinine (P<0.01), the correlation coefficient was 0.567 (positive correlation); There was also a significant correlation between DPR and creatinine (P<0.01), and the correlation coefficient was -0.808 (negative correlation). The correlations of DPR with triglyceride and total cholesterol were significantly negative correlated (P<0.05), with -0.201 and -0.210 of correlation coefficients, respectively. The remaining indexes did not show any correlation with ALSFRS-R and DPR.
Conclusions 1. Uric acid and creatinine of ALS patients were lower than that in healthy people. Creatine kinase, triglyceride, total cholesterol, HCY, and cystatin C in ALS patients were higher than those in health controls. There were significant metabolic abnormalities in ALS patients.
2. Creatinine level is an independent risk factor affecting ALSFRS-R. The creatinine and total cholesterol levels are also the independent risk factors affecting DPR. Creatinine and total cholesterol levels could be used as reliable indicators to evaluate the ALSFRS-R and DPR of ALS patients.

scholarly journals Eye Movement Abnormalities in Amyotrophic Lateral Sclerosis

2021 ◽  
Author(s):  
Xintong Guo ◽  
Xiaoxuan Liu ◽  
Shan Ye ◽  
Xiangyi Liu ◽  
Xu Yang ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Eye Movement ◽  
Smooth Pursuit ◽  
Oculomotor System ◽  
Control Group ◽  
Clinical Markers ◽  
Square Wave ◽  
Movement Parameters ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background and Purpose It is generally believed that eye movements are completely spared in amyotrophic lateral sclerosis (ALS). Although a series of eye movement abnormalities has been recognized in recent years, the findings are highly controversial, and the corresponding pattern has not yet been established. Furthermore, bulbar disabilities should be considered in relation to eye movement abnormalities. The present study aimed to determine whether eye movement abnormalities are present in ALS and, if so, to investigate their characteristics and their association with bulbar disability in ALS patients. Methods Patients with clinically definite, probable or laboratory-supported probable ALS (n=60) and a control group composed of their caregivers (n=30) underwent clinical assessments and standardized evaluations of the oculomotor system using videonystagmography. The gaze test, reflexive saccade test and smooth pursuit test were administered to all subjects. Results Eye movement abnormalities such as square-wave jerks, abnormal cogwheeling during smooth pursuit, and saccade hypometria were observed in ALS patients. Square-wave jerks (p<0.001) and abnormal cogwheeling during smooth pursuit (p=0.001) were more frequently observed in ALS patients than in the control subjects. In subgroup analyses, square-wave jerks (p=0.004) and abnormal cogwheeling during smooth pursuit (p=0.031) were found to be more common in ALS patients with bulbar involvement (n=44) than in those without bulbar involvement (n=16). There were no significant differences in the investigated eye movement parameters between bulbar-onset (n=12) and spinal-onset patients (n=48). Conclusion ALS patients showed a range of eye movement abnormalities, affecting mainly the ocular fixation and smooth pursuit systems. These abnormalities were observed more common in the ALS patients with bulbar involvement. Our pioneering study indicates that the region of involvement could better indicate the pathophysiological essence of the abnormalities than the type of onset pattern in ALS. Eye movement abnormalities may be potential clinical markers for objectively evaluating upper brainstem or supratentorial cerebral lesion neurodegeneration in ALS.

scholarly journals Magnetic resonance findings in amyotrophic lateral sclerosis using a spin echo magnetization transfer sequence: preliminary report

1999 ◽  
Vol 57 (4) ◽  
pp. 912-915 ◽  
Author(s):  
ANTÔNIO JOSÉ DA ROCHA ◽  
ANTONIO CARLOS MARTINS MAIA JUNIOR ◽  
ROBERTO GOMES NOGUEIRA ◽  
HENRIQUE MANOEL LEDERMAN
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Magnetic Resonance ◽  
Preliminary Report ◽  
Corticospinal Tract ◽  
Spin Echo ◽  
Magnetization Transfer ◽  
Control Group ◽  
Spin Echo Sequence ◽  
Lateral Sclerosis

We present the magnetic resonance (MR) findings of five patients with amyotrophic lateral sclerosis (ALS) using a spin-echo sequence with an additional magnetization transfer (MT) pulse on T1-weighted images (T1 SE/MT). These findings were absent in the control group and consisted of hyperintensity of the corticospinal tract. Moreover we discuss the principles and the use of this fast but simple MR technique in the diagnosis of ALS

scholarly journals Design and analysis of a clinical trial using previous trials as historical control

2019 ◽  
Vol 16 (5) ◽  
pp. 531-538 ◽  
Author(s):  
David Alan Schoenfeld ◽  
Dianne M Finkelstein ◽  
Eric Macklin ◽  
Neta Zach ◽  
David L Ennist ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Amyotrophic Lateral Sclerosis ◽  
Sample Size ◽  
Standard Error ◽  
Controlled Trial ◽  
Sample Size Calculation ◽  
Control Group ◽  
Controlled Trials ◽  
Number Of Patients ◽  
Lateral Sclerosis

Background/AimsFor single arm trials, a treatment is evaluated by comparing an outcome estimate to historically reported outcome estimates. Such a historically controlled trial is often analyzed as if the estimates from previous trials were known without variation and there is no trial-to-trial variation in their estimands. We develop a test of treatment efficacy and sample size calculation for historically controlled trials that considers these sources of variation.MethodsWe fit a Bayesian hierarchical model, providing a sample from the posterior predictive distribution of the outcome estimand of a new trial, which, along with the standard error of the estimate, can be used to calculate the probability that the estimate exceeds a threshold. We then calculate criteria for statistical significance as a function of the standard error of the new trial and calculate sample size as a function of difference to be detected. We apply these methods to clinical trials for amyotrophic lateral sclerosis using data from the placebo groups of 16 trials.ResultsWe find that when attempting to detect the small to moderate effect sizes usually assumed in amyotrophic lateral sclerosis clinical trials, historically controlled trials would require a greater total number of patients than concurrently controlled trials, and only when an effect size is extraordinarily large is a historically controlled trial a reasonable alternative. We also show that utilizing patient level data for the prognostic covariates can reduce the sample size required for a historically controlled trial.ConclusionThis article quantifies when historically controlled trials would not provide any sample size advantage, despite dispensing with a control group.

scholarly journals The adverse effect of wearing a face mask during the COVID-19 pandemic and benefits of wearing transparent face masks and using clear speech on speech intelligibility

2021 ◽  
Author(s):  
Hoyoung Yi ◽  
Ashly Pingsterhaus ◽  
Woonyoung Song
Keyword(s):  
Adverse Effect ◽  
Background Noise ◽  
Visual Information ◽  
Speech Intelligibility ◽  
Visual Cues ◽  
Word Identification ◽  
Signal To Noise Ratio ◽  
Face Mask ◽  
Presentation Mode ◽  
Clear Speech

The coronavirus pandemic has resulted in recommended/required use of a face mask in public. The use of a face mask compromises communication, especially in the presence of competing noise. It is crucial to measure potential adverse effect(s) of wearing face masks on speech intelligibility in communication contexts where excessive background noise occurs to lead to solutions for this communication challenge. Accordingly, effects of wearing transparent face masks and using clear speech to support better verbal communication was evaluated here. We evaluated listener word identification scores in the following four conditions: (1) type of masking (i.e., no mask, transparent mask, and disposable paper mask), (2) presentation mode (i.e., auditory only and audiovisual), (3) speaker speaking style (i.e., conversational speech and clear speech), and (4) with two types of background noise (i.e., speech shaped noise and four-talker babble at negative 5 signal to noise ratio levels). Results showed that in the presence of noise, listeners performed less well when the speaker wore a disposable paper mask or a transparent mask compared to wearing no mask. Listeners correctly identified more words in the audiovisual when listening to clear speech. Results indicate the combination of face masks and the presence of background noise impact speech intelligibility negatively for listeners. Transparent masks facilitate the ability to understand target sentences by providing visual information. Use of clear speech was shown to alleviate challenging communication situations including lack of visual cues and reduced acoustic signal.

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