ArticleNeurology2019
Spinal cord α-synuclein deposition associated with myoclonus in patients with MSA-C.
Article in Neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.
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Who cites it
7 citing papers in PubMed, 1 synthesis or guideline pooled it, 13 citations in OpenAlex.
- Tremor in multiple system atrophy: a systematic literature review.Journal of neural transmission (Vienna, Austria : 1996) · 2026Pooled it
- Conducting molybdenum sulfide/graphene oxide/polyvinyl alcohol nanocomposite hydrogel for repairing spinal cord injury.Journal of nanobiotechnology · 2022Article
- Heterogeneity of Multiple System Atrophy: An Update.Biomedicines · 2022Review
- Clinical neurophysiology of Parkinson's disease and parkinsonism.Clinical neurophysiology practice · 2022Review
- Neuropathology of multiple system atrophy: Kurt Jellinger`s legacy.Journal of neural transmission (Vienna, Austria : 1996) · 2021Review
- An update on MSA: premotor and non-motor features open a window of opportunities for early diagnosis and intervention.Journal of neurology · 2020Review
- AAV-Mediated GALC Gene Therapy Rescues Alpha-Synucleinopathy in the Spinal Cord of a Leukodystrophic Lysosomal Storage Disease Mouse Model.Frontiers in cellular neuroscience · 2020Article
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Authors and funding
6 authors at 3 institutions in 1 country.
Funding
Abstract
objectiveTo test the hypothesis that myoclonus in patients with multiple system atrophy with predominant cerebellar ataxia (MSA-C) is associated with a heavier burden of α-synuclein deposition in the motor regions of the spinal cord, we compared the degree of α-synuclein deposition in spinal cords of 3 patients with MSA-C with myoclonus and 3 without myoclonus.
methodsAll human tissue was obtained by the Massachusetts General Hospital Department of Pathology with support from and according to neuropathology guidelines of the Massachusetts Alzheimer's Disease Research Center. Tissue was stained with Luxol fast blue and hematoxylin & eosin for morphologic evaluation, and with a mouse monoclonal antibody to α-synuclein and Vectastain DAB kit. Images of the spinal cord sections were digitized using a 10× objective lens. Grayscale versions of these images were transferred to ImageJ software for quantitative analysis of 8 different regions of interest (ROIs) in the spinal cord: dorsal column, anterior white column, left and right dorsal horns, left and right anterior horns, and left and right lateral corticospinal tracts. A mixed-effect, multiple linear regression model was constructed to determine if patients with and without myoclonus had significantly different distributions of α-synuclein deposition across the various ROIs.
resultsPatients with myoclonus had more α-synuclein in the anterior horns (
conclusionsIn MSA-C, myoclonus appears to be associated with a higher burden of α-synuclein deposition within spinal cord motor regions. Future studies with more patients will be needed to confirm these findings.
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