scholarly journals Complement activation at the motor end-plates in amyotrophic lateral sclerosis

2016 ◽  
Vol 13 (1) ◽  
Author(s):  
Nawal Bahia El Idrissi ◽  
Sanne Bosch ◽  
Valeria Ramaglia ◽  
Eleonora Aronica ◽  
Frank Baas ◽  
...  
2020 ◽  
Vol 34 (6) ◽  
pp. 7225-7233
Author(s):  
Tanya S. McDonald ◽  
Pamela A. McCombe ◽  
Trent M. Woodruff ◽  
John D. Lee

2011 ◽  
Vol 42 (3) ◽  
pp. 211-220 ◽  
Author(s):  
M. Sta ◽  
R.M.R. Sylva-Steenland ◽  
M. Casula ◽  
J.M.B.V. de Jong ◽  
D. Troost ◽  
...  

2008 ◽  
Vol 45 (16) ◽  
pp. 4163
Author(s):  
Isaac M. Chiu ◽  
Bela Kosaras ◽  
Stefanos A. Tsiftsoglou ◽  
Ming Zhang ◽  
Timothy K. Vartanian ◽  
...  

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.


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