Intact single muscle fibres from SOD1 G93A amyotrophic lateral sclerosis mice display preserved specific force, fatigue resistance and training‐like adaptations

2019 ◽  
Vol 597 (12) ◽  
pp. 3133-3146 ◽  
Author(s):  
Arthur J. Cheng ◽  
Ilary Allodi ◽  
Thomas Chaillou ◽  
Maja Schlittler ◽  
Niklas Ivarsson ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Fatigue Resistance ◽  
Muscle Fibres ◽  
Single Muscle ◽  
Specific Force ◽  
And Training ◽  
Lateral Sclerosis

scholarly journals An Online Tool to Inform and Educate Caregivers on Amyotrophic Lateral Sclerosis (ALS)

2021 ◽  
Author(s):  
Maria Giannaki ◽  
Parisis Gallos ◽  
Joseph Liaskos ◽  
Spyros Zogas ◽  
John Mantas
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Evaluation Process ◽  
Content Management ◽  
Design Development ◽  
Structured Interviews ◽  
Online Tool ◽  
Training Materials ◽  
And Training ◽  
Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is the most common Motor Neuron Disease. This paper presents the design, development, and evaluation of an online tool that provides information and training materials to caregivers about ALS, to promote health care and quality of life of patients. To collect the appropriate content, a literature review was conducted, and a Content Management System (CMS) was used for the development of the tool. For its evaluation, reliability, acceptance, effectiveness and usefulness were examined through semi-structured interviews. The online tool was positively evaluated by the caregivers, who participated in the evaluation process.

Properties of slow- and fast-twitch muscle fibres in a mouse model of amyotrophic lateral sclerosis

2005 ◽  
Vol 15 (5) ◽  
pp. 377-388 ◽  
Author(s):  
Julie D. Atkin ◽  
Rachel L. Scott ◽  
Jan M. West ◽  
Elizabeth Lopes ◽  
Alvin K.J. Quah ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Muscle Fibres ◽  
Fast Twitch Muscle ◽  
Fast Twitch ◽  
Lateral Sclerosis

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.

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