FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3150267725> ?p ?o ?g. }

• W3150267725 abstract "Introduction: Nusinersen is a recent promising therapy approved for the treatment of spinal muscular atrophy (SMA), a rare disease characterized by the degeneration of alpha motor neurons (αMN) in the spinal cord (SC) leading to progressive muscle atrophy and dysfunction. Muscle and cervical SC quantitative magnetic resonance imaging (qMRI) has never been used to monitor drug treatment in SMA. The aim of this pilot study is to investigate whether qMRI can provide useful biomarkers for monitoring treatment efficacy in SMA. Methods: Three adult SMA 3a patients under treatment with nusinersen underwent longitudinal clinical and qMRI examinations every 4 months from baseline to 21-month follow-up. The qMRI protocol aimed to quantify thigh muscle fat fraction (FF) and water-T2 (w-T2) and to characterize SC volumes and microstructure. Eleven healthy controls underwent the same SC protocol (single time point). We evaluated clinical and imaging outcomes of SMA patients longitudinally and compared SC data between groups transversally. Results: Patient motor function was stable, with only Patient 2 showing moderate improvements. Average muscle FF was already high at baseline (50%) and progressed over time (57%). w-T2 was also slightly higher than previously published data at baseline and slightly decreased over time. Cross-sectional area of the whole SC, gray matter (GM), and ventral horns (VHs) of Patients 1 and 3 were reduced compared to controls and remained stable over time, while GM and VHs areas of Patient 2 slightly increased. We found altered diffusion and magnetization transfer parameters in SC structures of SMA patients compared to controls, thus suggesting changes in tissue microstructure and myelin content. Conclusion: In this pilot study, we found a progression of FF in thigh muscles of SMA 3a patients during nusinersen therapy and a concurrent slight reduction of w-T2 over time. The SC qMRI analysis confirmed previous imaging and histopathological studies suggesting degeneration of αMN of the VHs, resulting in GM atrophy and demyelination. Our longitudinal data suggest that qMRI could represent a feasible technique for capturing microstructural changes induced by SMA in vivo and a candidate methodology for monitoring the effects of treatment, once replicated on a larger cohort." @default.
• W3150267725 created "2021-04-13" @default.
• W3150267725 creator A5001734076 @default.
• W3150267725 creator A5006836444 @default.
• W3150267725 creator A5033822190 @default.
• W3150267725 creator A5034573269 @default.
• W3150267725 creator A5037442059 @default.
• W3150267725 creator A5038742855 @default.
• W3150267725 creator A5042582841 @default.
• W3150267725 creator A5042811968 @default.
• W3150267725 creator A5048499258 @default.
• W3150267725 creator A5051669233 @default.
• W3150267725 creator A5055648634 @default.
• W3150267725 creator A5068790660 @default.
• W3150267725 creator A5070364341 @default.
• W3150267725 creator A5072844704 @default.
• W3150267725 creator A5083732015 @default.
• W3150267725 creator A5090162853 @default.
• W3150267725 creator A5090359104 @default.
• W3150267725 date "2021-03-29" @default.
• W3150267725 modified "2023-10-14" @default.
• W3150267725 title "Pilot Study on Quantitative Cervical Cord and Muscular MRI in Spinal Muscular Atrophy: Promising Biomarkers of Disease Evolution and Treatment?" @default.
• W3150267725 cites W1702490096 @default.
• W3150267725 cites W1871235281 @default.
• W3150267725 cites W1963427860 @default.
• W3150267725 cites W1963604250 @default.
• W3150267725 cites W1985112961 @default.
• W3150267725 cites W2000290854 @default.
• W3150267725 cites W2000520189 @default.
• W3150267725 cites W2007271427 @default.
• W3150267725 cites W2024317059 @default.
• W3150267725 cites W2028464246 @default.
• W3150267725 cites W2028861985 @default.
• W3150267725 cites W2070008028 @default.
• W3150267725 cites W2076586285 @default.
• W3150267725 cites W2088007629 @default.
• W3150267725 cites W2094514781 @default.
• W3150267725 cites W2105034316 @default.
• W3150267725 cites W2122258381 @default.
• W3150267725 cites W2125953041 @default.
• W3150267725 cites W2127890285 @default.
• W3150267725 cites W2133032830 @default.
• W3150267725 cites W2143197369 @default.
• W3150267725 cites W2207653270 @default.
• W3150267725 cites W2309887726 @default.
• W3150267725 cites W2328069846 @default.
• W3150267725 cites W2474514554 @default.
• W3150267725 cites W2523445319 @default.
• W3150267725 cites W2524030778 @default.
• W3150267725 cites W2528264181 @default.
• W3150267725 cites W2528727157 @default.
• W3150267725 cites W2540377240 @default.
• W3150267725 cites W2557310973 @default.
• W3150267725 cites W2579663022 @default.
• W3150267725 cites W2584307689 @default.
• W3150267725 cites W2606866670 @default.
• W3150267725 cites W2755662383 @default.
• W3150267725 cites W2762683804 @default.
• W3150267725 cites W2766426594 @default.
• W3150267725 cites W2787482106 @default.
• W3150267725 cites W2897476004 @default.
• W3150267725 cites W2903344422 @default.
• W3150267725 cites W2912007568 @default.
• W3150267725 cites W2938579539 @default.
• W3150267725 cites W2963963144 @default.
• W3150267725 cites W2971756469 @default.
• W3150267725 cites W2990689917 @default.
• W3150267725 cites W2997839939 @default.
• W3150267725 cites W3002862034 @default.
• W3150267725 cites W3004624483 @default.
• W3150267725 cites W3008340394 @default.
• W3150267725 cites W3011851030 @default.
• W3150267725 cites W3039651163 @default.
• W3150267725 cites W3043287113 @default.
• W3150267725 cites W3045989829 @default.
• W3150267725 cites W3084730147 @default.
• W3150267725 doi "https://doi.org/10.3389/fneur.2021.613834" @default.
• W3150267725 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/8039452" @default.
• W3150267725 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33854470" @default.
• W3150267725 hasPublicationYear "2021" @default.
• W3150267725 type Work @default.
• W3150267725 sameAs 3150267725 @default.
• W3150267725 citedByCount "7" @default.
• W3150267725 countsByYear W31502677252021 @default.
• W3150267725 countsByYear W31502677252022 @default.
• W3150267725 countsByYear W31502677252023 @default.
• W3150267725 crossrefType "journal-article" @default.
• W3150267725 hasAuthorship W3150267725A5001734076 @default.
• W3150267725 hasAuthorship W3150267725A5006836444 @default.
• W3150267725 hasAuthorship W3150267725A5033822190 @default.
• W3150267725 hasAuthorship W3150267725A5034573269 @default.
• W3150267725 hasAuthorship W3150267725A5037442059 @default.
• W3150267725 hasAuthorship W3150267725A5038742855 @default.
• W3150267725 hasAuthorship W3150267725A5042582841 @default.
• W3150267725 hasAuthorship W3150267725A5042811968 @default.
• W3150267725 hasAuthorship W3150267725A5048499258 @default.
• W3150267725 hasAuthorship W3150267725A5051669233 @default.
• W3150267725 hasAuthorship W3150267725A5055648634 @default.
• W3150267725 hasAuthorship W3150267725A5068790660 @default.
• W3150267725 hasAuthorship W3150267725A5070364341 @default.

• next