Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy
Objective To characterize and quantify peripheral nerve lesions and muscle degeneration in clinically, genetically, and electrophysiologically well-classified, nonpediatric patients with 5q-linked spinal muscular atrophy (SMA) by high-resolution magnetic resonance neurography (MRN). Methods Thirty-o...
Gespeichert in:
| Hauptverfasser: | , , , , , , , , , , , |
|---|---|
| Dokumenttyp: | Article (Journal) |
| Sprache: | Englisch |
| Veröffentlicht: |
July 10, 2019
|
| In: |
Neurology
Year: 2019, Jahrgang: 93, Heft: 7, Pages: E653-E664 |
| ISSN: | 1526-632X |
| DOI: | 10.1212/WNL.0000000000007945 |
| Online-Zugang: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1212/WNL.0000000000007945
|
| Verfasserangaben: | Jennifer Kollmer, Tim Hilgenfeld, Andreas Ziegler, Afshin Saffari, Georges Sam, John M. Hayes, Adriana Pietsch, Marie Jost, Sabine Heiland, Martin Bendszus, Wolfgang Wick, Markus Weiler |
MARC
| LEADER | 00000caa a22000002c 4500 | ||
|---|---|---|---|
| 001 | 1693336111 | ||
| 003 | DE-627 | ||
| 005 | 20220818021128.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 200326s2019 xx |||||o 00| ||eng c | ||
| 024 | 7 | |a 10.1212/WNL.0000000000007945 |2 doi | |
| 035 | |a (DE-627)1693336111 | ||
| 035 | |a (DE-599)KXP1693336111 | ||
| 035 | |a (OCoLC)1341311183 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rda | ||
| 041 | |a eng | ||
| 084 | |a 33 |2 sdnb | ||
| 100 | 1 | |a Hayes, Jennifer |d 1981- |e VerfasserIn |0 (DE-588)139430822 |0 (DE-627)703322737 |0 (DE-576)31172843X |4 aut | |
| 245 | 1 | 0 | |a Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy |c Jennifer Kollmer, Tim Hilgenfeld, Andreas Ziegler, Afshin Saffari, Georges Sam, John M. Hayes, Adriana Pietsch, Marie Jost, Sabine Heiland, Martin Bendszus, Wolfgang Wick, Markus Weiler |
| 264 | 1 | |c July 10, 2019 | |
| 300 | |a 12 | ||
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a Computermedien |b c |2 rdamedia | ||
| 338 | |a Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Gesehen am 26.03.2020 | ||
| 520 | |a Objective To characterize and quantify peripheral nerve lesions and muscle degeneration in clinically, genetically, and electrophysiologically well-classified, nonpediatric patients with 5q-linked spinal muscular atrophy (SMA) by high-resolution magnetic resonance neurography (MRN). Methods Thirty-one adult patients with genetically confirmed 5q-linked SMA types II, IIIa, and IIIb and 31 age- and sex-matched healthy volunteers were prospectively investigated. All patients received neurologic, physiotherapeutic, and electrophysiologic assessments. MRN at 3.0T with anatomic coverage from the lumbosacral plexus and proximal thigh down to the tibiotalar joint was performed with dual-echo 2D relaxometry sequences with spectral fat saturation and a 3D T2-weighted inversion recovery sequence. Detailed quantification of nerve injury by morphometric and microstructural MRN markers and qualitative classification of fatty muscle degeneration were conducted. Results Established clinical scores and compound muscle action potentials discriminated well between the 3 SMA types. MRN revealed that peroneal and tibial nerve cross-sectional area (CSA) at the thigh and lower leg level as well as spinal nerve CSA were markedly decreased throughout all 3 groups, indicating severe generalized peripheral nerve atrophy. While peroneal and tibial nerve T2 relaxation time was distinctly increased at all analyzed anatomic regions, the proton spin density was clearly decreased. Marked differences in fatty muscle degeneration were found between the 3 groups and for all analyzed compartments. Conclusions MRN detects and quantifies peripheral nerve involvement in SMA types II, IIIa, and IIIb with high sensitivity in vivo. Quantitative MRN parameters (T2 relaxation time, proton spin density, CSA) might serve as novel imaging biomarkers in SMA to indicate early microstructural nerve tissue changes in response to treatment. | ||
| 650 | 4 | |a challenges | |
| 650 | 4 | |a familial amyloid polyneuropathy | |
| 650 | 4 | |a nerve injury | |
| 650 | 4 | |a nusinersen | |
| 650 | 4 | |a scale | |
| 650 | 4 | |a sham control | |
| 650 | 4 | |a smn1 | |
| 650 | 4 | |a therapy | |
| 650 | 4 | |a time | |
| 650 | 4 | |a version | |
| 700 | 1 | |a Hilgenfeld, Tim |d 1986- |e VerfasserIn |0 (DE-588)1106261917 |0 (DE-627)862954398 |0 (DE-576)473950316 |4 aut | |
| 700 | 1 | |a Ziegler, Andreas |d 1979- |e VerfasserIn |0 (DE-588)134123530 |0 (DE-627)560894201 |0 (DE-576)278444253 |4 aut | |
| 700 | 1 | |a Saffari, Afshin |d 1988- |e VerfasserIn |0 (DE-588)1076753302 |0 (DE-627)835291251 |0 (DE-576)445631406 |4 aut | |
| 700 | 1 | |a Sam, Georges |d 1966- |e VerfasserIn |0 (DE-588)131798162 |0 (DE-627)514262990 |0 (DE-576)29875875X |4 aut | |
| 700 | 1 | |a Hayes, John M. |e VerfasserIn |4 aut | |
| 700 | 1 | |a Pietsch, Adriana |e VerfasserIn |4 aut | |
| 700 | 1 | |a Jost, Marie |e VerfasserIn |4 aut | |
| 700 | 1 | |a Heiland, Sabine |e VerfasserIn |0 (DE-588)106732626X |0 (DE-627)818624450 |0 (DE-576)426561368 |4 aut | |
| 700 | 1 | |a Bendszus, Martin |e VerfasserIn |0 (DE-588)1032676426 |0 (DE-627)738634131 |0 (DE-576)175567697 |4 aut | |
| 700 | 1 | |a Wick, Wolfgang |d 1970- |e VerfasserIn |0 (DE-588)120297736 |0 (DE-627)080586929 |0 (DE-576)186221320 |4 aut | |
| 700 | 1 | |a Weiler, Markus |d 1972- |e VerfasserIn |0 (DE-588)123920256 |0 (DE-627)706474236 |0 (DE-576)293943079 |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Neurology |d Philadelphia, Pa. : Wolters Kluwer, 1951 |g 93(2019), 7, Seite E653-E664 |h Online-Ressource |w (DE-627)302718524 |w (DE-600)1491874-2 |w (DE-576)079876609 |x 1526-632X |7 nnas |a Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy |
| 773 | 1 | 8 | |g volume:93 |g year:2019 |g number:7 |g pages:E653-E664 |g extent:12 |a Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy |
| 856 | 4 | 0 | |u https://doi.org/10.1212/WNL.0000000000007945 |x Verlag |x Resolving-System |z lizenzpflichtig |3 Volltext |
| 951 | |a AR | ||
| 992 | |a 20200326 | ||
| 993 | |a Article | ||
| 994 | |a 2019 | ||
| 998 | |g 123920256 |a Weiler, Markus |m 123920256:Weiler, Markus |d 50000 |e 50000PW123920256 |k 0/50000/ |p 12 |y j | ||
| 998 | |g 120297736 |a Wick, Wolfgang |m 120297736:Wick, Wolfgang |d 910000 |d 911100 |e 910000PW120297736 |e 911100PW120297736 |k 0/910000/ |k 1/910000/911100/ |p 11 | ||
| 998 | |g 1032676426 |a Bendszus, Martin |m 1032676426:Bendszus, Martin |d 910000 |d 911100 |e 910000PB1032676426 |e 911100PB1032676426 |k 0/910000/ |k 1/910000/911100/ |p 10 | ||
| 998 | |g 106732626X |a Heiland, Sabine |m 106732626X:Heiland, Sabine |d 910000 |d 911100 |e 910000PH106732626X |e 911100PH106732626X |k 0/910000/ |k 1/910000/911100/ |p 9 | ||
| 998 | |g 131798162 |a Sam, Georges |m 131798162:Sam, Georges |d 910000 |d 911100 |e 910000PS131798162 |e 911100PS131798162 |k 0/910000/ |k 1/910000/911100/ |p 5 | ||
| 998 | |g 1076753302 |a Saffari, Afshin |m 1076753302:Saffari, Afshin |d 910000 |d 910500 |e 910000PS1076753302 |e 910500PS1076753302 |k 0/910000/ |k 1/910000/910500/ |p 4 | ||
| 998 | |g 134123530 |a Ziegler, Andreas |m 134123530:Ziegler, Andreas |d 910000 |d 910500 |e 910000PZ134123530 |e 910500PZ134123530 |k 0/910000/ |k 1/910000/910500/ |p 3 | ||
| 998 | |g 1106261917 |a Hilgenfeld, Tim |m 1106261917:Hilgenfeld, Tim |d 910000 |d 911100 |e 910000PH1106261917 |e 911100PH1106261917 |k 0/910000/ |k 1/910000/911100/ |p 2 | ||
| 998 | |g 139430822 |a Hayes, Jennifer |m 139430822:Hayes, Jennifer |d 910000 |d 911100 |e 910000PH139430822 |e 911100PH139430822 |k 0/910000/ |k 1/910000/911100/ |p 1 |x j | ||
| 999 | |a KXP-PPN1693336111 |e 3613857901 | ||
| BIB | |a Y | ||
| SER | |a journal | ||
| JSO | |a {"id":{"eki":["1693336111"],"doi":["10.1212/WNL.0000000000007945"]},"name":{"displayForm":["Jennifer Kollmer, Tim Hilgenfeld, Andreas Ziegler, Afshin Saffari, Georges Sam, John M. Hayes, Adriana Pietsch, Marie Jost, Sabine Heiland, Martin Bendszus, Wolfgang Wick, Markus Weiler"]},"title":[{"title":"Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy","title_sort":"Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophy"}],"note":["Gesehen am 26.03.2020"],"language":["eng"],"type":{"bibl":"article-journal","media":"Online-Ressource"},"relHost":[{"type":{"media":"Online-Ressource","bibl":"periodical"},"language":["eng"],"note":["Gesehen am 12.12.23"],"id":{"zdb":["1491874-2"],"issn":["1526-632X"],"eki":["302718524"]},"part":{"pages":"E653-E664","issue":"7","extent":"12","text":"93(2019), 7, Seite E653-E664","year":"2019","volume":"93"},"title":[{"subtitle":"official journal of the American Academy of Neurology","title":"Neurology","title_sort":"Neurology"}],"origin":[{"publisher":"Wolters Kluwer ; Ovid ; Lippincott Williams & Wilkins","dateIssuedKey":"1951","dateIssuedDisp":"1951-","publisherPlace":"Philadelphia, Pa. ; [Erscheinungsort nicht ermittelbar] ; Hagerstown, Md."}],"pubHistory":["1.1951 -"],"disp":"Quantitative MR neurography biomarkers in 5q-linked spinal muscular atrophyNeurology","physDesc":[{"extent":"Online-Ressource"}],"recId":"302718524"}],"person":[{"display":"Hayes, Jennifer","family":"Hayes","given":"Jennifer","role":"aut"},{"given":"Tim","role":"aut","family":"Hilgenfeld","display":"Hilgenfeld, Tim"},{"role":"aut","given":"Andreas","family":"Ziegler","display":"Ziegler, Andreas"},{"display":"Saffari, Afshin","role":"aut","given":"Afshin","family":"Saffari"},{"display":"Sam, Georges","role":"aut","given":"Georges","family":"Sam"},{"display":"Hayes, John M.","given":"John M.","role":"aut","family":"Hayes"},{"given":"Adriana","role":"aut","family":"Pietsch","display":"Pietsch, Adriana"},{"family":"Jost","role":"aut","given":"Marie","display":"Jost, Marie"},{"display":"Heiland, Sabine","family":"Heiland","given":"Sabine","role":"aut"},{"family":"Bendszus","role":"aut","given":"Martin","display":"Bendszus, Martin"},{"family":"Wick","role":"aut","given":"Wolfgang","display":"Wick, Wolfgang"},{"family":"Weiler","role":"aut","given":"Markus","display":"Weiler, Markus"}],"origin":[{"dateIssuedDisp":"July 10, 2019","dateIssuedKey":"2019"}],"recId":"1693336111","physDesc":[{"extent":"12 S."}]} | ||
| SRT | |a HAYESJENNIQUANTITATI1020 | ||