Quantitative 1H and 23Na muscle MRI in Facioscapulohumeral muscular dystrophy patients

Abstract

Objective Our aim was to assess the role of quantitative 1H and 23Na MRI methods in providing imaging biomarkers of disease activity and severity in patients with Facioscapulohumeral muscular dystrophy (FSHD). Methods We imaged the lower leg muscles of 19 FSHD patients and 12 controls with a multimodal MRI protocol to obtain STIR-T2w images, fat fraction (FF), water T2 (wT2), water T1 (wT1), tissue sodium concentration (TSC), and intracellular-weighted sodium signal (inversion recovery (IR) and triple quantum filter (TQF) sequence). In addition, the FSHD patients underwent muscle strength testing. Results Imaging biomarkers related with water mobility (wT1 and wT2) and ion homeostasis (TSC, IR, TQF) were increased in muscles of FSHD patients. Muscle groups with FF > 10% had higher wT2, wT1, TSC, IR, and TQF values than muscles with FF < 10%. Muscles with FF < 10% resembled muscles of healthy controls for these MRI disease activity measures. However, wT1 was increased in few muscles without fat replacement. Furthermore, few STIR-negative muscles (n = 11/76) exhibited increased wT1, TSC, IR or TQF. Increased wT1 as well as 23Na signals were also present in muscles with normal wT2. Muscle strength was related to the mean FF and all imaging biomarkers of tibialis anterior except wT2 were correlated with dorsal flexion. Conclusion The newly evaluated imaging biomarkers related with water mobility (wT1) and ion homeostasis (TSC, IR, TQF) showed different patterns compared to the established markers like FF in muscles of FSHD patients. These quantitative biomarkers could thus contain valuable complementary information for the early characterization of disease progression.

Primary hyperaldosteronism induces congruent alterations of sodium homeostasis in different skeletal muscles: a 23Na-MRI study

“…As we focused on a disease which assumedly affects the body’s skeletal sodium storage in a global manner, our results do not apply for diseases with definite inhomogeneous affection of the skeletal muscle system, and, hence, sodium homeostasis, as demonstrated, for example, in neuromuscular diseases affecting singular muscles or compartments ( 5 , 9 ). Examinations of the thoracic organs (e.g.…”

“…in neuromuscular diseases affecting singular muscles or compartments. (5,9) Examinations of the thoracic organs (e.g., lung or heart) usually benefit from prospective or retrospective gating or some method of post-processing that is applied to compensate for motion (artefacts). (10) As our patients were examined in prone position, movement of the pectoral muscle was minimized.…”

Spatial Distribution of Muscular Effects of Acute Whole-Body Electromyostimulation at the Mid-Thigh and Lower Leg—A Pilot Study Applying Magnetic Resonance Imaging

“…The depth of the edema (Table 3) ranged from superficial for the m. vastus lateralis in Figure 2 center MRI scan to maximum depth for the m. vastus intermedialis in Figure 2 upper MRI scan. Edema volume at the mid-thigh according to the scale of Gerhalter et al [20] was consistently ≤33% of the entire muscle. In summary, we determined grade one muscle damage in superficial but also deeperlying muscle layers/groups, not only but particularly for the hamstrings (i.e., m. biceps femoris, semimembranosus, semitendinosus); however, we failed to observe a consistently uniform pattern of muscle damage among the eight participants.…”

“…For the semi-quantitative analysis, a radiologist with 8 years' experience in reading musculoskeletal MRIs evaluated all muscles both at the upper and lower leg. Signal intensity in TIRM images representing intramuscular edema was rated using a modified four-point scale from 0 to 3, based on the scale described by Gerhalter et al [20] and by Wang et al [21]: 0-normal appearance; 1-areas of increased signal intensity, 1%-33% of the entire muscle volume; 2-areas of increased signal intensity, 34%-66% of the volume; 3-areas of increased signal intensity, 67%-100% of the volume. All imaging slices of each muscle were considered for the grading.…”

Sodium in the dermis colocates to glycosaminoglycan scaffold, with diminishment in type 2 diabetes mellitus

“…Similarly, insertion of a TQ filter (TQF) into sodium MRI protocols suppresses all signals except TQ-tagged 23 Na; recorded MRI depicts bound sodium only. TQF MRI and TQ-TPPI MRS are well developed and understood ( 23 , 25 ) and have been utilized previously in biomedical studies to distinguish between sodium within the ICF and ECF in cell suspensions spectroscopically ( 26 ) and visualized using TQF 23 Na MRI in the brain ( 27 , 28 ), in the muscle ( 29 ), and in the cartilage ( 30 ). Furthermore, standard gradient echo (GE) 23 Na MRI protocols largely suppress signals arising from bound sodium due to its inherent fast T2 relaxation.…”