scholarly journals Different sensorimotor mechanism in fast and slow progression amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Qianwen Li ◽  
Wenjia Zhu ◽  
Xinmei Wen ◽  
Zhenxiang Zang ◽  
Yuwei Da ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Slow Progression ◽  
Lateral Sclerosis

scholarly journals Two cases of familial amyotrophic lateral sclerosis with a SOD1L126S mutation showing high age at onset and slow progression

2010 ◽  
Vol 50 (3) ◽  
pp. 163-167 ◽  
Author(s):  
Tomo Iwashima ◽  
Takahisa Tateishi ◽  
Ryo Yamasaki ◽  
Kyoko Motomura ◽  
Yasumasa Ohyagi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Age At Onset ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Slow Progression ◽  
High Age ◽  
Lateral Sclerosis

A case of amyotrophic lateral sclerosis with a very slow progression over 44 years

2001 ◽  
Vol 11 (4) ◽  
pp. 414-416 ◽  
Author(s):  
K. Grohme ◽  
M. v. Maravic ◽  
T. Gasser ◽  
G.D. Borasio
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Slow Progression ◽  
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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