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Muscle damage, edema, and fat infiltration are hallmarks of a range of neuromuscular diseases. The T2 of water, T2,w, in muscle lengthens with both myocellular damage and inflammation and is typically measured using multiple spin-echo or Carr-Purcell-Meiboom-Gill acquisitions. However, microscopic fat infiltration in neuromuscular diseases prevents accurate T2,w quantitation as the longer T2 of fat, T2,f, masks underlying changes in the water component. Fat saturation can be inconsistent across the imaging volume and removes valuable physiological fat information. A new method is presented that combines iterative decomposition of water and fat with echo asymmetry and least squares estimation with a Carr-Purcell-Meiboom-Gill-sequence. The sequence results in water and fat separated images at each echo time for use in T2,w and T2,f quantification. With knowledge of the T2,w and T2,f, a T2-corrected fat fraction map can also be calculated. Monte-Carlo simulations and measurements in phantoms, volunteers, and a patient with inclusion body myositis are demonstrated. In healthy volunteers, uniform T2,w and T2-corrected fat fraction maps are present within all muscle groups. However, muscle-specific patterns of fat infiltration and edema are evident in inclusion body myositis, which demonstrates the power of separating and quantifying the fat and water components. Magn Reson Med, 2011. © 2011 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Purpose: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a treatable, immune-mediated condition characterised by progressive or relapsing motor and sensory neurological deficits. The diagnosis is based on a combination of clinical, neurophysiological and supportive criteria, but can be challenging. In this study, we quantified the diameter and cross-sectional area of the lumbosacral nerve roots, and explored the imaging characteristics of the sciatic nerves, in patients with CIDP versus healthy controls using MRI., Methods: MRI of the lumbosacral plexus and both thighs was performed at 3 T. Orthogonal diameter and cross-sectional area of the lumbosacral nerve roots were measured, along with sciatic nerve cross-sectional area at the mid-thigh level. The MRI appearance of the sciatic nerves was also evaluated qualitatively. All measurements were performed by an observer blinded to the diagnosis., Results: 10 patients with CIDP and 10 healthy controls (age and sex-matched) were studied. Lumbosacral nerve root diameter and cross-sectional area were significantly increased in patients with CIDP compared to controls (mean diameter 6.0 ± 1.1 mm vs 4.8 ± 0.3 mm; p = 0.006), with a high sensitivity (89 %) and specificity (90 %) on ROC analysis. Sciatic nerve cross sectional area was also significantly increased in the CIDP group, and was accompanied by qualitative MRI changes., Conclusions: Quantitative MRI reveals significant hypertrophy of the lumbosacral nerve roots and sciatic nerves in patients with CIDP compared to controls. This study provides further evidence for the inclusion of lumbosacral nerve root and sciatic nerve hypertrophy on MRI as a supportive feature in the diagnostic criteria for CIDP., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Quantifying muscle water T 2 (T 2 -water) independently of intramuscular fat content is essential in establishing T 2 -water as an outcome measure for imminent new therapy trials in neuromuscular diseases. IDEAL-CPMG combines chemical shift fat-water separation with T 2 relaxometry to obtain such a measure. Here we evaluate the reproducibility and B 1 sensitivity of IDEAL-CPMG T 2 -water and fat fraction (f.f.) values in healthy subjects, and demonstrate the potential of the method to quantify T 2 -water variation in diseased muscle displaying varying degrees of fatty infiltration. The calf muscles of 11 healthy individuals (40.5 ± 10.2 years) were scanned twice at 3 T with an inter-scan interval of 4 weeks using IDEAL-CPMG, and 12 patients with hypokalemic periodic paralysis (HypoPP) (42.3 ± 11.5 years) were also imaged. An exponential was fitted to the signal decay of the separated water and fat components to determine T 2 -water and the fat signal amplitude muscle regions manually segmented. Overall mean calf-level muscle T 2 -water in healthy subjects was 31.2 ± 2.0 ms, without significant inter-muscle differences (p = 0.37). Inter-subject and inter-scan coefficients of variation were 5.7% and 3.2% respectively for T 2 -water and 41.1% and 15.4% for f.f. Bland-Altman mean bias and ±95% coefficients of repeatability were for T 2 -water (0.15, -2.65, 2.95) ms and f.f. (-0.02, -1.99, 2.03)%. There was no relationship between T 2 -water (ρ = 0.16, p = 0.07) or f.f. (ρ = 0.03, p = 0.7761) and B 1 error or any correlation between T 2 -water and f.f. in the healthy subjects (ρ = 0.07, p = 0.40). In HypoPP there was a measurable relationship between T 2 -water and f.f. (ρ = 0.59, p < 0.001). IDEAL-CPMG provides a feasible way to quantify T 2 -water in muscle that is reproducible and sensitive to meaningful physiological changes without post hoc modeling of the fat contribution. In patients, IDEAL-CPMG measured elevations in T 2 -water and f.f. while showing a weak relationship between these parameters, thus showing promise as a practical means of quantifying muscle water in patient populations., (© 2016 The Authors. NMR in Biomedicine published by John Wiley & Sons Ltd.)

Introduction: In this study we investigated muscle magnetic resonance imaging in congenital myasthenic syndromes (CMS)., Methods: Twenty-six patients with 9 CMS subtypes and 10 controls were imaged. T1-weighted (T1w) and short-tau inversion recovery (STIR) 3-Tesla MRI images obtained at thigh and calf levels were scored for severity., Results: Overall mean the T1w score was increased in GFPT1 and DPAGT1 CMS. T1w scans of the AChR-deficiency, COLQ, and CHAT subjects were indistinguishable from controls. STIR images from CMS patients did not differ significantly from those of controls. Mean T1w score correlated with age in the CMS cohort., Conclusions: MRI appearances ranged from normal to marked abnormality. T1w images seem to be especially abnormal in some CMS caused by mutations of proteins involved in the glycosylation pathway. A non-selective pattern of fat infiltration or a normal-appearing scan in the setting of significant clinical weakness should suggest CMS as a potential diagnosis. Muscle MRI could play a role in differentiating CMS subtypes. Muscle Nerve 54: 211-219, 2016., (© 2016 The Authors. Muscle & Nerve Published by Wiley Periodicals, Inc.)

Purpose: To compare the influence of two limb positions and slice prescription using scout-image-based and surface-anatomy-based methods on the reproducibility of quantitative MRI of lower-limb muscles., Materials and Methods: Ten healthy subjects were scanned at 3 Tesla with a two-dimensional turbo spin-echo T1-weighted acquisition. Imaging was performed at thigh and calf level in two subject limb positions and independently repeated by a second operator. Regions-of-interest (ROI) were drawn on three muscles at thigh and calf levels on axial slices at fixed distance from the knee joint and at a level determined by surface anatomy., Results: Test-retest reliability of muscle cross-sectional area and ROI area overlap were similar for both limb positioning methods. Changing limb position between scans reduced ROI overlap (P < 0.01). Scout-image-based slice prescription resulted in narrower limits of agreement and higher intraclass correlation coefficients compared with surface-anatomy-based slice prescription., Conclusion: Slice prescription based on fixed distance from the knee joint provided superior reproducibility of slice location than a surface anatomy-based method and should be used for longitudinal quantitative MRI studies. Exact subject positioning will depend on scanner and coil configuration, but should be consistent through a longitudinal study., (Copyright © 2013 Wiley Periodicals, Inc.)

We assessed the presence, frequency and pattern of MRI abnormalities in non-dystrophic myotonia patients. We reviewed T1-weighted and STIR (short-tau-inversion-recovery) 3T MRI sequences of lower limb muscles at thigh and calf level in 21 patients with genetically confirmed non-dystrophic myotonia: 11 with CLCN1 mutations and 10 with SCN4A mutations, and 19 healthy volunteers. The MRI examinations of all patients showed hyperintensity within muscles on either T1-weighted or STIR images. Mild extensive or marked T1-weighted changes were noted in 10/21 patients and no volunteers. Muscles in the thigh were equally likely to be affected but in the calf there was sparing of tibialis posterior. Oedema was common in calf musculature especially in the medial gastrocnemius with STIR hyperintensity observed in 18/21 patients. In 10/11 CLCN1 patients this included a previously unreported "central stripe", also present in 3/10 SCN4A patients but no volunteers. Degree of fatty infiltration correlated with age (rho=0.46, p<0.05). Muscle MRI is frequently abnormal in non-dystrophic myotonia providing evidence of fatty infiltration and/or oedema. The pattern is distinct from other myotonic disorders; in particular the "central stripe" has not been reported in other conditions. Correlations with clinical parameters suggest a potential role for MRI as a biomarker., (Copyright © 2013 The Authors. Published by Elsevier B.V. All rights reserved.)