Observational studyJournal of neurology2020
Longitudinal multi-modal muscle-based biomarker assessment in motor neuron disease.
Observational study in Journal of neurology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 11 papers, 2 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
11 citing papers in PubMed, 2 syntheses or guidelines pooled it, 28 citations in OpenAlex.
- Muscle MRI and Muscle Ultrasound Applications in MND/ALS: Academic Insights and Clinical Opportunities.European journal of neurology · 2026Pooled it
- The evolving role of surface electromyography in amyotrophic lateral sclerosis: A systematic review.Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology · 2020Pooled it
- Screen, Sample, Stratify: Biomarkers and Machine Learning Compress Dementia Pathways.Biomedicines · 2026Article
- Magnetic resonance imaging applications to amyotrophic lateral sclerosis beyond the central nervous system.Frontiers in neurology · 2026Review
- Muscle MRI quantifies disease progression in amyotrophic lateral sclerosis.Journal of neurology, neurosurgery, and psychiatry · 2025Article
- The central pattern of weakness of ALS: Morphological correlates in whole-body muscle MRI.Annals of clinical and translational neurology · 2024Article
- Prognostic Usefulness of Motor Unit Number Index (MUNIX) in Patients Newly Diagnosed with Amyotrophic Lateral Sclerosis.Journal of clinical medicine · 2023Article
- BDNF-dependent modulation of axonal transport is selectively impaired in ALS.Acta neuropathologica communications · 2022Article
- VoxelHop: Successive Subspace Learning for ALS Disease Classification Using Structural MRI.IEEE journal of biomedical and health informatics · 2022Article
- Confocal Endomicroscopy of Neuromuscular Junctions Stained with Physiologically Inert Protein Fragments of Tetanus Toxin.Biomolecules · 2021Article
- Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis.Frontiers in neurology · 2021Article
Corrections and comments
- Erratum issued
Authors and funding
9 authors at 2 institutions in 1 country.
Funding
Abstract
backgroundClinical phenotypic heterogeneity represents a major barrier to trials in motor neuron disease (MND) and objective surrogate outcome measures are required, especially for slowly progressive patients. We assessed responsiveness of clinical, electrophysiological and radiological muscle-based assessments to detect MND-related progression. MATERIALS AND
methodsA prospective, longitudinal cohort study of 29 MND patients and 22 healthy controls was performed. Clinical measures, electrophysiological motor unit number index/size (MUNIX/MUSIX) and relative T2- and diffusion-weighted whole-body muscle magnetic resonance (MR) were assessed three times over 12 months. Multi-variable regression models assessed between-group differences, clinico-electrophysiological associations, and longitudinal changes. Standardized response means (SRMs) assessed sensitivity to change over 12 months.
resultsMND patients exhibited 18% higher whole-body mean muscle relative T2-signal than controls (95% CI 7-29%, p < 0.01), maximal in leg muscles (left tibialis anterior 71% (95% CI 33-122%, p < 0.01). Clinical and electrophysiological associations were evident. By 12 months, 16 patients had died or could not continue. In the remainder, relative T2-signal increased over 12 months by 14-29% in right tibialis anterior, right quadriceps, bilateral hamstrings and gastrocnemius/soleus (p < 0.01), independent of onset-site, and paralleled progressive weakness and electrophysiological loss of motor units. Highest clinical, electrophysiological and radiological SRMs were found for revised ALS-functional rating scale scores (1.22), tibialis anterior MUNIX (1.59), and relative T2-weighted leg muscle MR (right hamstrings: 0.98), respectively. Diffusion MR detected minimal changes.
conclusionMUNIX and relative T2-weighted MR represent objective surrogate markers of progressive denervation in MND. Radiological changes were maximal in leg muscles, irrespective of clinical onset-site.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.