Sonographic and 3T-MRI-based evaluation of the tongue in ALS

Hensiek, Nathalie; Schreiber, Frank; Wimmer, Thomas; Kaufmann, Jörn; Machts, Judith; Fahlbusch, Laura; Garz, Cornelia; Vogt, Susanne; Prudlo, Johannes; Dengler, Reinhard; Petri, Susanne; Nestor, Peter J.; Vielhaber, Stefan; Schreiber, Stefanie

doi:10.1016/j.nicl.2020.102233

Cited by 16 publications

(12 citation statements)

References 31 publications

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“…Among these 28 articles, there were 14 MND/ ALS (18-31), 13 SMA (32-44) and three SBMA (20,29,45) patient groups. There were eight multiple-region (21,(24)(25)(26)28,29,37,45), 13 lower-limb (19,20,(32)(33)(34)(35)(36)38,(40)(41)(42)(43)(44), one paraspinal (30), three bulbar (18,22,23) and three whole-body (27,31,39) studies. These papers are summarized in Tables 1 and 2.…”

Section: Resultsmentioning

confidence: 99%

“…Studies in motor neuron diseases have applied quantitative (18,19,(22)(23)(24)29,(31)(32)(33)35,39,(42)(43)(44)(45), qualitative (18,20,21,25,26,(28)(29)(30)(32)(33)(34)(36)(37)(38)(39)(40)(41)45), and semi-quantitative (27,31) techniques, often in combination, and imaging protocols vary between studies. Most qualitative studies used the fivepoint Mercuri scale, which grades change from an early moth-eaten appearance to muscle replacement by connective tissue and fat (46).…”

Section: Discussionmentioning

confidence: 99%

“…Frequent issues identified were omission of a control group (32,33,36,37,(39)(40)(41)43), lack of blinding (18)(19)(20)23,31,(34)(35)(36)(39)(40)(41)(42)(43)45) and inadequate ascertainment of disease status (23,25,40). Only four studies justified the sample size (22,27,31,38). More than 20% cohort attrition was reported in 5/9 longitudinal studies, typical in MND research.…”

Section: Assessment Of Biasmentioning

confidence: 99%

“…Finally, in 2020, tongue volume and mean T1 intensity were assessed in large cohorts of 185 MND patients and 104 healthy controls (22). Tongue T1 intensity was lower in bulbar-onset than limb-onset patients (p < .001) and correlated with ALSFRSr bulbar sub-scores (r¼-0.2, p ¼ .02), and larger or more ellipsoidal tongues at baseline predicted a slower decline in ALSFRSr.…”

Section: Mndmentioning

confidence: 99%

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Muscle MRI in motor neuron diseases: a systematic review

Kriss

Jenkins

2021

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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Objective: To summarize applications of muscle magnetic resonance imaging (MRI) in cross-sectional assessment and longitudinal monitoring of motor neuron diseases and evaluate associations with clinical assessment techniques. Methods: PubMed and Scopus were searched for research published up to May 2021 relating to muscle MRI in motor neuron diseases, according to predefined inclusion and exclusion criteria. Studies were systematically appraised for bias and data were extracted for discussion. Results: Twenty-eight papers met inclusion criteria. The studies assessed muscle T1-and T2-weighted signal, diffusion, muscle volume, and fat infiltration, employing quantitative, qualitative, and semi-quantitative approaches. Various regions of interest were considered; changes in thigh and calf muscles were most frequently reported. Preliminary evidence of concordance between clinical and radiological findings and utility as an objective longitudinal biomarker is emerging. Conclusion: Muscle MRI appears a promising objective, versatile, and practical biomarker to assess motor neuron diseases.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Assessment Of Biasmentioning

confidence: 99%

Section: Mndmentioning

confidence: 99%

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Muscle MRI in motor neuron diseases: a systematic review

Kriss

Jenkins

2021

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

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“…Ultrasound has been shown to have interrater and intrarater reliability when measuring longitudinal muscle loss and represents a potential marker of progression (50). When combined with MRI, tongue ultrasound predicted bulbar progression and, interestingly, overall motor function (51).…”

Section: Imagingmentioning

confidence: 99%

Biomarkers in amyotrophic lateral sclerosis: a review of new developments

Verber

Shaw²

2020

Current Opinion in Neurology

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Longitudinal Quantitative MRI Provides Responsive Outcome Measures for Early and Late Muscle Changes in ALS

Diaz,

Thornton,

Wastling

et al. 2024

Muscle and Nerve

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Introduction/AimsStudies have demonstrated the potential of muscle MRIs to measure disease progression in ALS. However, the responsiveness and utility of quantitative muscle MRIs in an ALS clinical trial remain unknown. This study aimed to determine the responsiveness of quantitative muscle MRIs to measure disease progression in ALS.MethodsLongitudinal quantitative muscle MRIs were obtained in an ALS study that delivered human neural progenitor cells to the spinal cord (NCT02943850). Participants underwent MRIs at baseline, 1, 3, 6, 9, and 12 months. MRI measures included fat fraction (ff), water T2 (T2m), cross‐sectional area (CSA), and remaining muscle area (RMA). Non‐MRI measures included strength via Accurate Test of Limb Isometric Strength (ATLIS) and the ALSFRS‐R. Standardized response means (SRM) were calculated at 1, 3, 6, and 12 months.ResultsSignificant increases in muscle FF and decreases in CSA and RMA were seen as early as 1 month from baseline. At 6 months, the most responsive measures were muscle FF (SRMthigh = 1.85, SRMcalf = 1.39), T2m (SRMthigh = 1.2, SRMcalf = 1.71), CSA (SRMthigh = −1.58, SRMcalf = −1.14), RMA (SRMthigh = −1.77, SRMcalf = −1.28), and strength tested via ATLIS (SRMknee extension = −1.79, SRMknee flexion = −1.3). The ALSFRS‐R was the least responsive at 6 months (SRM = −0.85). Muscle FF and T2m correlated with ALSFRS‐R leg subscores and MRI measures demonstrated varying degrees of correlation with strength.DiscussionHigh responsiveness and low variability make quantitative muscle MRI a novel and complementary outcome measure for ALS clinical trials.

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Sonographic and 3T-MRI-based evaluation of the tongue in ALS

Cited by 16 publications

References 31 publications

Muscle MRI in motor neuron diseases: a systematic review

Muscle MRI in motor neuron diseases: a systematic review

Biomarkers in amyotrophic lateral sclerosis: a review of new developments

Longitudinal Quantitative MRI Provides Responsive Outcome Measures for Early and Late Muscle Changes in ALS

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