Dysferlin-deficient Muscular Dystrophy: Gadofluorine M Suitability at MR Imaging in a Mouse Model1
S Schmidt, A Vieweger, M Obst, S Mueller, V Gross… - Radiology, 2009 - pubs.rsna.org
S Schmidt, A Vieweger, M Obst, S Mueller, V Gross, M Gutberlet, J Steinbrink, S Taubert…
Radiology, 2009•pubs.rsna.orgPurpose: To compare the usefulness of gadofluorine M with that of Gadomer in assessment
of dysferlin-deficient muscular dystrophy at 7.0-T magnetic resonance (MR) imaging.
Materials and Methods: All experiments were approved by local review boards. SJL/J mice
(n= 24) with dysferlin-deficient muscular dystrophy and C57BL/6 control mice (n= 24) were
imaged at 12–15 weeks (young) or older than 30 weeks (old) by using dynamic contrast
material–enhanced imaging with inversion-prepared steady-state free-precession sequence …
of dysferlin-deficient muscular dystrophy at 7.0-T magnetic resonance (MR) imaging.
Materials and Methods: All experiments were approved by local review boards. SJL/J mice
(n= 24) with dysferlin-deficient muscular dystrophy and C57BL/6 control mice (n= 24) were
imaged at 12–15 weeks (young) or older than 30 weeks (old) by using dynamic contrast
material–enhanced imaging with inversion-prepared steady-state free-precession sequence …
Purpose: To compare the usefulness of gadofluorine M with that of Gadomer in assessment of dysferlin-deficient muscular dystrophy at 7.0-T magnetic resonance (MR) imaging.
Materials and Methods: All experiments were approved by local review boards. SJL/J mice (n = 24) with dysferlin-deficient muscular dystrophy and C57BL/6 control mice (n = 24) were imaged at 12–15 weeks (young) or older than 30 weeks (old) by using dynamic contrast material–enhanced imaging with inversion-prepared steady-state free-precession sequence before, during, and after administration of gadofluorine M at 2 μmol or Gadomer at 4 μmol intravenously. After imaging, regions of interest were determined from the upper extremity and left ventricular chamber; fractional extravascular extracellular volume, ve, and permeability surface tissue density product, PSρ, were measured by using a two-compartment pharmacokinetic model. The natural history of muscular dystrophy was assessed histologically in 70 mice (seven five-mouse groups each of SJL/J mice and of control mice) at 4-week intervals from 8 to 32 weeks. In addition, three SJL/J mice and three control mice at age 33 weeks were sacrificed, and fluorescence microscopy was performed for visualization of intravenously administered carbocyanine-labeled gadofluorine M in muscle cells. Statistical analysis was performed by using the t test.
Results: Gadofluorine M enhancement was significantly greater in skeletal muscle of 30-week-old mice with dysferlin-deficient muscular dystrophy, compared with control mice. Gadofluorine M demonstrated both increased rate of enhancement (PSρ sec−1 ± standard error of the mean: 0.004e−4 ± 3 vs 0.002e−4 ± 3; P < .05) and increased level of enhancement (ve ± standard error of the mean: 0.035 ± 0.004 vs 0.019 ± 0.004; P < .05). Gadomer showed no differential enhancement in the two mouse groups. Histologic examination confirmed the presence of labeled gadofluorine M in muscle cells.
Conclusion: Gadofluorine M–enhanced MR imaging may be of value in monitoring dysferlin-deficient muscular dystrophy disease progression in this animal model and could prove to be a useful tool in following the course of chronic muscle diseases in humans.
Supplemental material: http://radiology.rsnajnls.org/cgi/content/full/2501080180/DC1
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