Dysferlin-deficient muscular dystrophy: gadofluorine M suitability at MR imaging in a mouse model.

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 micromol or Gadomer at 4 micromol intravenously. After imaging, regions of interest were determined from the upper extremity and left ventricular chamber; fractional extravascular extracellular volume, v(e), and permeability surface tissue density product, PS rho, 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 (PS rho sec(-1) +/- standard error of the mean: 0.004 e(-)(4) +/- 3 vs 0.002 e(-)(4) +/- 3; P < .05) and increased level of enhancement (v(e) +/- 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.

Imaging-therapy computed tomography with quasi-monochromatic X-rays.

INTRODUCTION: Computed tomography (CT) is a widespread and highly precise technique working in the energy range around 50-100 keV. For radiotherapy, however, the MeV energy range enables a better dose distribution. This gap between diagnosis and therapy can be overcome by the use of a modified CT machine in combination with heavy elements targeted to the tumour and used as photoelectric radiation enhancer. MATERIALS AND METHODS: The experimental setup consists of an X-ray optical module mounted at the exit of the X-ray tube of a clinical CT. The module converts the standard fan-shaped beam into a high intensity, monochromatized and focused beam. The radiation was characterized using an energy-dispersive detection system calibrated by synchrotron radiation and gel dosimetry. The photoelectric radiation enhancement for different elements was calculated and experimentally verified. RESULTS: The X-ray optical module filters selectively the energy of the tungsten K alpha-emission line (59.3 keV) with a full width at half maximum (FWHM) of 5 keV and focused the radiation onto a focal spot which coincides with the isocentre of the gantry. This results in a steep dose gradient at the centre of rotation qualified for locoregional radiation therapy. The photon energy of the quasi-monochromatic radiation agrees with the energy range of maximal photoelectric dose enhancement for gadolinium and iodine. CONCLUSION: An additional X-ray optical module optimized for targeted therapy and photoelectric dose enhancement allows the combination of diagnosis and radiotherapy on a clinical CT.