The multisystem degeneration amyotrophic lateral sclerosis - neuropathological staging and clinical translation

2018 ◽  
pp. 210-227
Author(s):  
Verde ◽  
K. Del Tredici ◽  
H. Braak
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Clinical Translation ◽  
Multisystem Degeneration ◽  
Lateral Sclerosis

Stem cell transplantation for amyotrophic lateral sclerosis: therapeutic potential and perspectives on clinical translation

2014 ◽  
Vol 71 (17) ◽  
pp. 3257-3268 ◽  
Author(s):  
Irene Faravelli ◽  
Giulietta Riboldi ◽  
Monica Nizzardo ◽  
Chiara Simone ◽  
Chiara Zanetta ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Therapeutic Potential ◽  
Clinical Translation ◽  
Lateral Sclerosis

Human stem cell models of neurodegeneration: From basic science of amyotrophic lateral sclerosis to clinical translation

2022 ◽  
Vol 29 (1) ◽  
pp. 11-35
Author(s):  
Elisa Giacomelli ◽  
Björn F. Vahsen ◽  
Elizabeth L. Calder ◽  
Yinyan Xu ◽  
Jakub Scaber ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Stem Cell ◽  
Basic Science ◽  
Clinical Translation ◽  
Human Stem Cell ◽  
Cell Models ◽  
Stem Cell Models ◽  
Lateral Sclerosis

scholarly journals Clinical translation of hepatocyte growth factor for amyotrophic lateral sclerosis

2012 ◽  
Vol 52 (11) ◽  
pp. 1214-1217
Author(s):  
Hitoshi Warita ◽  
Masaaki Kato ◽  
Naoki Suzuki ◽  
Yasuto Itoyama ◽  
Masashi Aoki
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Growth Factor ◽  
Hepatocyte Growth Factor ◽  
Clinical Translation ◽  
Hepatocyte Growth ◽  
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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