Reliability of maximal voluntary isometric contraction testing in a multicenter study of patients with amyotrophic lateral sclerosis

Background—Amyotrophic lateral sclerosis (ALS) is a devastating and untreatable motor neuron disease; smoking and alcohol drinking may impact its progression rate. Objective—To ascertain the influence of smoking and alcohol consumption on ALS progression rates. Methods—Cross-sectional multicenter study, including 241 consecutive patients (145 males); mean age at onset was 59.9 ± 11.8 years. Cigarette smoking and alcohol consumption data were collected at recruitment through a validated questionnaire. Patients were categorized into three groups according to ΔFS (derived from the ALS Functional Rating Scale-Revised and disease duration from onset): slow (n = 81), intermediate (80), and fast progressors (80). Results—Current smokers accounted for 44 (18.3%) of the participants, former smokers accounted for 10 (4.1%), and non-smokers accounted for 187 (77.6%). The age of ALS onset was lower in current smokers than non-smokers, and the ΔFS was slightly, although not significantly, higher for smokers of >14 cigarettes/day. Current alcohol drinkers accounted for 147 (61.0%) of the participants, former drinkers accounted for 5 (2.1%), and non-drinkers accounted for 89 (36.9%). The log(ΔFS) was weakly correlated only with the duration of alcohol consumption (p = 0.028), but not with the mean number of drinks/day or the drink-years. Conclusions: This cross-sectional multicenter study suggested a possible minor role for smoking in worsening disease progression. A possible interaction with alcohol drinking was suggested.

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Progressive Neurochemical Abnormalities in Cognitive and Motor Subgroups of Amyotrophic Lateral Sclerosis: A Prospective Multicenter Study

Neurology ◽

10.1212/wnl.0000000000012367 ◽

2021 ◽

pp. 10.1212/WNL.0000000000012367

Author(s):

Daniel Ta ◽

Abdullah Ishaque ◽

Ojas Srivastava ◽

Chris Hanstock ◽

Peter Seres ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Prefrontal Cortex ◽

Motor Cortex ◽

Disease Progression ◽

Multicenter Study ◽

Rapid Disease Progression ◽

Prospective Longitudinal ◽

Lateral Sclerosis ◽

Over Time ◽

Cerebral Degeneration

OBJECTIVE:To evaluate progressive cerebral degeneration in amyotrophic lateral sclerosis (ALS) by assessing alterations in N-acetylaspartate (NAA) ratios in the motor and prefrontal cortex within clinical subgroups of ALS.METHODS:Seventy-six ALS patients and 59 healthy controls were enrolled in a prospective, longitudinal, multicenter study in the Canadian ALS Neuroimaging Consortium (CALSNIC). Participants underwent serial clinical evaluations and MR spectroscopy at baseline, 4 and 8 months using a harmonized protocol across 5 centers. NAA ratios were quantified in the motor cortex and prefrontal cortex. Patients were stratified into subgroups based on disease progression rate, upper motor neuron (UMN) signs, and cognitive status. Linear mixed models were used for baseline and longitudinal comparisons of NAA metabolite ratios.RESULTS:Patients with ALS had reduced NAA ratios in the motor cortex at baseline (P < 0.001). Ratios were lower in those with more rapid disease progression and greater UMN signs (P < 0.05). A longitudinal decline in NAA ratios was observed in the motor cortex in the rapid progressing (P < 0.01) and high UMN burden (P < 0.01) cohorts. The severity of UMN signs did not change significantly over time. NAA ratios were reduced in the prefrontal cortex only in cognitively impaired patients (P < 0.05); prefrontal cortex metabolites did not change over time.CONCLUSIONS:Progressive degeneration of the motor cortex in ALS is associated with more aggressive clinical presentations These findings provide biological evidence of variable spatial and temporal cerebral degeneration linked to the disease heterogeneity of ALS. The use of standardized imaging protocols may have a role to play in clinical trials for patient selection or subgrouping.CLASSIFICATION OF EVIDENCE:This study provides Class II evidence that MRS NAA metabolite ratios of the motor cortex are associated with more rapid disease progression and greater UMN signs in patients with ALS.

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Repeated courses of granulocyte colony-stimulating factor in amyotrophic lateral sclerosis: Clinical and biological results from a prospective multicenter study

Muscle & Nerve ◽

10.1002/mus.21851 ◽

2011 ◽

Vol 43 (2) ◽

pp. 189-195 ◽

Cited By ~ 40

Author(s):

Adriano Chiò ◽

Gabriele Mora ◽

Vincenzo La Bella ◽

Claudia Caponnetto ◽

Gianluigi Mancardi ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Multicenter Study ◽

Granulocyte Colony Stimulating Factor ◽

Colony Stimulating Factor ◽

Prospective Multicenter Study ◽

Stimulating Factor ◽

Lateral Sclerosis

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P08.2 A multicenter study on sensory potentials in amyotrophic lateral sclerosis

Clinical Neurophysiology ◽

10.1016/j.clinph.2006.06.294 ◽

2006 ◽

Vol 117 ◽

pp. 168-169

Author(s):

K. Pugdahl ◽

M. de Carvalho ◽

A. Fuglsang-Frederiksen ◽

B. Johnsen ◽

P. Fawcett ◽

...

Keyword(s):

Amyotrophic Lateral Sclerosis ◽

Multicenter Study ◽

Lateral Sclerosis

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Reduction rate of body mass index predicts prognosis for survival in amyotrophic lateral sclerosis: A multicenter study in Japan

Amyotrophic Lateral Sclerosis ◽

10.3109/17482968.2012.678366 ◽

2012 ◽

Vol 13 (4) ◽

pp. 363-366 ◽

Cited By ~ 56

Author(s):

Toshio Shimizu ◽

Utako Nagaoka ◽

Yuki Nakayama ◽

Akihiro Kawata ◽

Chiharu Kugimoto ◽

...

Keyword(s):

Body Mass Index ◽

Amyotrophic Lateral Sclerosis ◽

Body Mass ◽

Multicenter Study ◽

Reduction Rate ◽

Lateral Sclerosis

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Automated Acoustic Analysis of Oral Diadochokinesis to Assess Bulbar Motor Involvement in Amyotrophic Lateral Sclerosis

Journal of Speech Language and Hearing Research ◽

10.1044/2019_jslhr-19-00178 ◽

2020 ◽

Vol 63 (1) ◽

pp. 59-73 ◽

Cited By ~ 3

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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