scholarly journals The Effects of Symptom Onset Location on Automatic Amyotrophic Lateral Sclerosis Detection Using the Correlation Structure of Articulatory Movements

2021 ◽  
pp. 1-11
Author(s):  
Alan Wisler ◽  
Kristin Teplansky ◽  
Daragh Heitzman ◽  
Jun Wang
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Regression Analysis ◽  
Symptom Onset ◽  
Correlation Structure ◽  
Speaking Rate ◽  
Articulatory Movements ◽  
Significant Difference ◽  
Bulbar Onset ◽  
Lateral Sclerosis ◽  
Onset Location

Purpose Kinematic measurements of speech have demonstrated some success in automatic detection of early symptoms of amyotrophic lateral sclerosis (ALS). In this study, we examined how the region of symptom onset (bulbar vs. spinal) affects the ability of data-driven models to detect ALS. Method We used a correlation structure of articulatory movements combined with a machine learning model (i.e., artificial neural network) to detect differences between people with ALS and healthy controls. The performance of this system was evaluated separately for participants with bulbar onset and spinal onset to examine how region of onset affects classification performance. We then performed a regression analysis to examine how different severity measures and region of onset affects model performance. Results The proposed model was significantly more accurate in classifying the bulbar-onset participants, achieving an area under the curve of 0.809 relative to the 0.674 achieved for spinal-onset participants. The regression analysis, however, found that differences in classifier performance across participants were better explained by their speech performance (intelligible speaking rate), and no significant differences were observed based on region of onset when intelligible speaking rate was accounted for. Conclusions Although we found a significant difference in the model's ability to detect ALS depending on the region of onset, this disparity can be primarily explained by observable differences in speech motor symptoms. Thus, when the severity of speech symptoms (e.g., intelligible speaking rate) was accounted for, symptom onset location did not affect the proposed computational model's ability to detect ALS.

scholarly journals Prevalence and Factors Related to Pathological Laughter and Crying in Patients With Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Qian-Qian Wei ◽  
Ruwei Ou ◽  
Junyu Lin ◽  
Lingyu Zhang ◽  
Yanbing Hou ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Regression Analysis ◽  
Rating Scale ◽  
Cox Proportional Hazards ◽  
Disease Stage ◽  
Female Patients ◽  
Pathological Laughter ◽  
Bulbar Onset ◽  
Late Disease Stage ◽  
Lateral Sclerosis

Objective: This study aimed to explore the prevalence and clinical correlates of pathological laughter and crying (PLC) in patients with amyotrophic lateral sclerosis (ALS).Methods: A total of 1,031 ALS patients were enrolled between August 2012 and August 2019. The PLC was recorded by a face-to-face interview. Other characteristics of patients, including depression, anxiety, cognition, and behavior function, were also evaluated. The potential associated factors of PLC were explored using forward binary regression analysis. Survival was analyzed in groups using propensity score matching (PSM) and Cox proportional hazards models.Results: The prevalence of PLC was 11.4% in all patients at baseline. Bulbar-onset and female patients had higher prevalence of PLC. The multivariate regression analysis indicated that PLC in ALS was associated with bulbar onset (p < 0.001), late disease stage (p < 0.001), and higher score in the Hamilton Depression Rating Scale (HDRS) (p = 0.012). The higher score of HDRS was significantly and independently associated with PLC occurrence in bulbar-onset patients (p = 0.032). The late disease stage was related to PLC occurrence in spinal-onset patients (p < 0.001). After comparison with matched pairs by using PSM, PLC at baseline had no impact on survival.Conclusion: PLC was not uncommon in ALS, especially in bulbar-onset and female patients. We highlighted that the emotional state other than cognitive function had possible relationship with PLC in ALS.

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.

Age at symptom onset influences cortical thinning distribution and survival in amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Pilar M. Ferraro ◽  
Corrado Cabona ◽  
Giuseppe Meo ◽  
Claudia Rolla-Bigliani ◽  
Lucio Castellan ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Symptom Onset ◽  
Cortical Thinning ◽  
Lateral Sclerosis

scholarly journals Utility of Transcranial Magnetic Simulation in Studying Upper Motor Neuron Dysfunction in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 11 (7) ◽  
pp. 906
Author(s):  
Nimeshan Geevasinga ◽  
Mehdi Van den Bos ◽  
Parvathi Menon ◽  
Steve Vucic
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Cortical Excitability ◽  
Muscular Atrophy ◽  
Close Relationship ◽  
Bulbar Onset ◽  
Als Patients ◽  
Transcranial Magnetic Simulation ◽  
Cortical Dysfunction ◽  
Lateral Sclerosis

Amyotrophic lateral sclerosis (ALS) is characterised by progressive dysfunction of the upper and lower motor neurons. The disease can evolve over time from focal limb or bulbar onset to involvement of other regions. There is some clinical heterogeneity in ALS with various phenotypes of the disease described, from primary lateral sclerosis, progressive muscular atrophy and flail arm/leg phenotypes. Whilst the majority of ALS patients are sporadic in nature, recent advances have highlighted genetic forms of the disease. Given the close relationship between ALS and frontotemporal dementia, the importance of cortical dysfunction has gained prominence. Transcranial magnetic stimulation (TMS) is a noninvasive neurophysiological tool to explore the function of the motor cortex and thereby cortical excitability. In this review, we highlight the utility of TMS and explore cortical excitability in ALS diagnosis, pathogenesis and insights gained from genetic and variant forms of the disease.

scholarly journals Can Executive Cognitive Measures Differentiate Between Patients with Spinal- and Bulbar-Onset Amyotrophic Lateral Sclerosis?

2012 ◽  
Vol 27 (3) ◽  
pp. 348-354 ◽  
Author(s):  
I. Zalonis ◽  
F. Christidi ◽  
G. Paraskevas ◽  
T. Zabelis ◽  
I. Evdokimidis ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Cognitive Measures ◽  
Bulbar Onset ◽  
Lateral Sclerosis

scholarly journals Syntactic Comprehension in Patients with Amyotrophic Lateral Sclerosis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Kentarou Yoshizawa ◽  
Nao Yasuda ◽  
Michinari Fukuda ◽  
Yumi Yukimoto ◽  
Mieko Ogino ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Executive Function ◽  
Sample Size ◽  
Executive Dysfunction ◽  
Frontal Assessment Battery ◽  
Auditory Comprehension ◽  
Assessment Battery ◽  
Visuospatial Function ◽  
Bulbar Onset ◽  
Lateral Sclerosis

Recent neuropsychological studies of patients with amyotrophic lateral sclerosis (ALS) have demonstrated that some patients have aphasic symptoms, including impaired syntactic comprehension. However, it is not known if syntactic comprehension disorder is related to executive and visuospatial dysfunction. In this study, we evaluated syntactic comprehension using the Syntax Test for Aphasia (STA) auditory comprehension task, frontal executive function using the Frontal Assessment Battery (FAB), visuospatial function using Raven’s Coloured Progressive Matrices (RCPM), and dementia using the Hasegawa Dementia Scale-Revised (HDS-R) in 25 patients with ALS. Of the 25 patients, 18 (72%) had syntactic comprehension disorder (STA score < IV), nine (36%) had frontal executive dysfunction (FAB score < 14), six (24%) had visuospatial dysfunction (RCPM score < 24), and none had dementia (HDS-R score < 20). Nine of the 18 patients with syntactic comprehension disorder (50%) passed the FAB and RCPM. Although sample size was small, these patients had a low STA score but normal FAB and RCPM score. All patients with bulbar onset ALS had syntactic comprehension disorder. These results indicate that it might be necessary to assess syntactic comprehension in patients with bulbar onset ALS. The implications of these findings are discussed in relation to the pathological continuum of ALS.

scholarly journals Motor neuron translatome reveals deregulation of SYNGR4 and PLEKHB1 in mutant TDP-43 amyotrophic lateral sclerosis models

2020 ◽  
Vol 29 (16) ◽  
pp. 2647-2661 ◽  
Author(s):  
Rita F Marques ◽  
Jan B Engler ◽  
Katrin Küchler ◽  
Ross A Jones ◽  
Thomas Lingner ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Motor Neuron ◽  
Symptom Onset ◽  
Rna Binding ◽  
Affinity Purification ◽  
Disease Onset ◽  
Motor Symptom ◽  
Pathological Feature ◽  
Lateral Sclerosis

Abstract Amyotrophic lateral sclerosis (ALS) is an incurable neurological disease with progressive loss of motor neuron (MN) function in the brain and spinal cord. Mutations in TARDBP, encoding the RNA-binding protein TDP-43, are one cause of ALS, and TDP-43 mislocalization in MNs is a key pathological feature of &gt;95% of ALS cases. While numerous studies support altered RNA regulation by TDP-43 as a major cause of disease, specific changes within MNs that trigger disease onset remain unclear. Here, we combined translating ribosome affinity purification (TRAP) with RNA sequencing to identify molecular changes in spinal MNs of TDP-43–driven ALS at motor symptom onset. By comparing the MN translatome of hTDP-43A315T mice to littermate controls and to mice expressing wild type hTDP-43, we identified hundreds of mRNAs that were selectively up- or downregulated in MNs. We validated the deregulated candidates Tex26, Syngr4, and Plekhb1 mRNAs in an independent TRAP experiment. Moreover, by quantitative immunostaining of spinal cord MNs, we found corresponding protein level changes for SYNGR4 and PLEKHB1. We also observed these changes in spinal MNs of an independent ALS mouse model caused by a different patient mutant allele of TDP-43, suggesting that they are general features of TDP-43-driven ALS. Thus, we identified SYNGR4 and PLEKHB1 to be deregulated in MNs at motor symptom onset in TDP-43-driven ALS models. This spatial and temporal pattern suggests that these proteins could be functionally important for driving the transition to the symptomatic phase of the disease.

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