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http://dx.doi.org/10.25673/38246
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DC Field | Value | Language |
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dc.contributor.author | Mensch, Alexander | - |
dc.contributor.author | Kraya, Torsten | - |
dc.contributor.author | Koester, Felicitas | - |
dc.contributor.author | Müller, Tobias | - |
dc.contributor.author | Stoevesandt, Dietrich | - |
dc.contributor.author | Zierz, Stephan | - |
dc.date.accessioned | 2021-09-01T09:23:07Z | - |
dc.date.available | 2021-09-01T09:23:07Z | - |
dc.date.issued | 2020 | - |
dc.identifier.uri | https://opendata.uni-halle.de//handle/1981185920/38489 | - |
dc.identifier.uri | http://dx.doi.org/10.25673/38246 | - |
dc.description.abstract | Objective: MATR3-associated distal myopathy is a rare distal myopathy predominantly affecting lower legs as well as wrist- and finger extensors. Whilst most distal myopathies are clinically and genetically well characterized, diagnosis often remains challenging. Pattern-based magnetic resonance imaging (MRI) approaches offer valuable additional information. However, a consistent pattern of muscular affection is missing for most distal myopathies. Thus, the aim of the present study was to establish a disease-specific pattern of muscular involvement in MATR3-associated distal myopathy using whole-body MRI. Methods: 15 patients (25–79 years of age, 7 female) with MATR3-associated distal myopathy were subjected to whole-body MRI. The grade of fatty involution for individual muscles was determined using Fischer-Grading. Results were compared to established MRI-patterns of other distal myopathies. Results: There was a predominant affection of the distal lower extremities. Lower legs showed a severe fatty infiltration, prominently affecting gastrocnemius and soleus muscle. In thighs, a preferential involvement of semimembranous and biceps femoris muscle was observed. Severe affection of gluteus minimus muscle as well as axial musculature, mainly affecting the thoracic segments, was seen. A sufficient discrimination to other forms of distal myopathy based solely on MRI-findings of the lower extremities was not possible. However, the inclusion of additional body parts seemed to yield specificity. Interpretation: Muscle MRI of patients with MATR3-associated distal myopathy revealed a distinct pattern of muscular involvement. The usage of whole-body muscle MRI provided valuable additional findings as compared to regular MRI of the lower extremities to improve distinction from other disease entities. | eng |
dc.description.sponsorship | Publikationsfond MLU | - |
dc.language.iso | eng | - |
dc.rights.uri | https://creativecommons.org/licenses/by/4.0/ | - |
dc.subject.ddc | 610 | - |
dc.title | Whole-body muscle MRI of patients with MATR3-associated distal myopathy reveals a distinct pattern of muscular involvement and highlights the value of whole-body examination | eng |
dc.type | Article | - |
local.versionType | publishedVersion | - |
local.bibliographicCitation.journaltitle | Journal of neurology | - |
local.bibliographicCitation.volume | 267 | - |
local.bibliographicCitation.pagestart | 2408 | - |
local.bibliographicCitation.pageend | 2420 | - |
local.bibliographicCitation.publishername | Steinkopff | - |
local.bibliographicCitation.publisherplace | [Darmstadt] | - |
local.bibliographicCitation.doi | 10.1007/s00415-020-09862-9 | - |
local.openaccess | true | - |
dc.identifier.ppn | 175558606X | - |
local.bibliographicCitation.year | 2020 | - |
cbs.sru.importDate | 2021-09-01T09:21:54Z | - |
local.bibliographicCitation | Enthalten in Journal of neurology - [Darmstadt] : Steinkopff, 1891 | - |
local.accessrights.dnb | free | - |
Appears in Collections: | Open Access Publikationen der MLU |
Files in This Item:
File | Description | Size | Format | |
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Mensch2020_Article_Whole-bodyMuscleMRIOfPatientsW.pdf | 4.93 MB | Adobe PDF | View/Open |