ArticlePloS one2016
Cervical Spinal Cord Atrophy Profile in Adult SMN1-Linked SMA.
Article in PloS one, 2016. 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 19 papers.
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Who cites it
19 citing papers in PubMed, 27 citations in OpenAlex.
- Longitudinal spinal cord MRI in adult 5q-SMA: biomarker and pathophysiological insights.Journal of neurology · 2026Article
- Quantitative cervical cord MRI in spinal muscular atrophy: a sensitive imaging biomarker of disease evolution and treatment.Journal of neurology · 2025Article
- Whole-body muscle MRI in patients with spinal muscular atrophy.Journal of neurology · 2025Article
- Spinal cord gray matter atrophy is associated with disability in spinal muscular atrophy.Journal of neurology · 2025Article
- Review
- A reassessment of spinal cord pathology in severe infantile spinal muscular atrophy: Reassessment of spinal cord pathology.Neuropathology and applied neurobiology · 2024Article
- Brain magnetic resonance imaging of patients with spinal muscular atrophy type 2 and 3.NeuroImage. Clinical · 2024Article
- Association between spinal muscular atrophy type and delayed diagnosis and the risk of spinal deformity in Indonesian patients.European journal of medical research · 2023Article
- Role of circulating biomarkers in spinal muscular atrophy: insights from a new treatment era.Frontiers in neurology · 2023Review
- Pilot Study on Quantitative Cervical Cord and Muscular MRI in Spinal Muscular Atrophy: Promising Biomarkers of Disease Evolution and Treatment?Frontiers in neurology · 2021Article
- MRI patterns of muscle involvement in type 2 and 3 spinal muscular atrophy patients.Journal of neurology · 2020Article
- Magnetic resonance imaging of the cervical spinal cord in spinal muscular atrophy.NeuroImage. Clinical · 2019Article
- Spinal Cord Imaging in Amyotrophic Lateral Sclerosis: Historical Concepts-Novel Techniques.Frontiers in neurology · 2019Review
- Cord cross-sectional area at foramen magnum as a correlate of disability in amyotrophic lateral sclerosis.European radiology experimental · 2018Article
- Mild SMN missense alleles are only functional in the presence of SMN2 in mammals.Human molecular genetics · 2018Article
- Spinal Cord Gray Matter Atrophy in Amyotrophic Lateral Sclerosis.AJNR. American journal of neuroradiology · 2018Article
- Biomarkers of Spinal and Bulbar Muscle Atrophy (SBMA): A Comprehensive Review.Frontiers in neurology · 2018Review
- Fully automated grey and white matter spinal cord segmentation.Scientific reports · 2016Article
- Article
Corrections and comments
- Erratum issued
Authors and funding
13 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeThe mechanisms underlying the topography of motor deficits in spinal muscular atrophy (SMA) remain unknown. We investigated the profile of spinal cord atrophy (SCA) in SMN1-linked SMA, and its correlation with the topography of muscle weakness. MATERIALS AND
methodsEighteen SMN1-linked SMA patients type III/V and 18 age/gender-matched healthy volunteers were included. Patients were scored on manual muscle testing and functional scales. Spinal cord was imaged using 3T MRI system. Radial distance (RD) and cord cross-sectional area (CSA) measurements in SMA patients were compared to those in controls and correlated with strength and disability scores.
resultsCSA measurements revealed a significant cord atrophy gradient mainly located between C3 and C6 vertebral levels with a SCA rate ranging from 5.4% to 23% in SMA patients compared to controls. RD was significantly lower in SMA patients compared to controls in the anterior-posterior direction with a maximum along C4 and C5 vertebral levels (p-values < 10-5). There were no correlations between atrophy measurements, strength and disability scores.
conclusionsSpinal cord atrophy in adult SMN1-linked SMA predominates in the segments innervating the proximal muscles. Additional factors such as neuromuscular junction or intrinsic skeletal muscle defects may play a role in more complex mechanisms underlying weakness in these patients.
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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.