The 6-minute walk test, motor function measure and quantitative thigh muscle MRI in Becker muscular dystrophy: A cross-sectional study

Fischer, Dirk; Hafner, Patricia; Rubino, Daniela; Schmid, Maurice; Neuhaus, Cornelia; Jung, Hans H.; Bieri, Oliver; Haas, Tanja; Gloor, M; Fischmann, Arne; Bonati, Leo H.

doi:10.1016/j.nmd.2016.04.009

Cited by 37 publications

(31 citation statements)

References 33 publications

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“…Muscle PDFF values have been shown to relate to muscle strength in healthy subjects47 and to clinical parameters in patients with knee osteoarthritis48 and rotator cuff pathologies 35. Muscle PDFF mapping has recently been introduced as a noninvasive biomarker for disease progression in muscle dystrophies and myopathies 24, 25, 26, 27. One recent study also explored the utility of PDFF to assess neurogenic muscle atrophy in patients with Charcot‐Marie‐Tooth Type 1 and found significantly higher fat fractions in calf but not thigh muscles 49.…”

Section: Discussionmentioning

confidence: 99%

“…35 Muscle PDFF mapping has recently been introduced as a noninvasive biomarker for disease progression in muscle dystrophies and myopathies. [24][25][26][27] One recent study also explored the utility of PDFF to assess neurogenic muscle atrophy in patients with Charcot-Marie-Tooth Type 1 and found significantly higher fat fractions in calf but not thigh muscles. 49 To remain within the boundaries of a feasible scanning time for patients (i.e., 20 min), our study focused on thigh muscles adjacent to and partially innervated by the sciatic nerve.…”

Section: Discussionmentioning

confidence: 99%

“…To explore changes in the muscle texture, we used a chemical shift encoding‐based water–fat separation technique to quantify the proton‐density fat fraction (PDFF) on a 0–100% scale. PDFF mapping has recently been introduced as a noninvasive biomarker for disease progression in different neuromuscular disorders including Charcot‐Marie‐Tooth (CMT) –Neuropathy 24, 25, 26, 27. We hypothesized that in CIDP, proximal nerve damage, and neurogenic muscle atrophy can be accurately visualized by these two techniques.…”

Section: Introductionmentioning

confidence: 99%

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MRI biomarkers of proximal nerve injury in CIDP

Lichtenstein

Sprenger

Weiss

et al. 2017

Ann Clin Transl Neurol

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ObjectiveTo evaluate the utility of nerve diffusion tensor imaging (DTI), nerve cross‐sectional area, and muscle magnetic resonance imaging (MRI) multiecho Dixon for assessing proximal nerve injury in chronic inflammatory demyelinating polyneuropathy (CIDP).MethodsIn this prospective observational cohort study, 11 patients with CIDP and 11 healthy controls underwent a multiparametric MRI protocol with DTI of the sciatic nerve and assessment of muscle proton‐density fat fraction of the biceps femoris and the quadriceps femoris muscles by multiecho Dixon MRI. Patients were longitudinally evaluated by MRI, clinical examination, and nerve conduction studies at baseline and after 6 months.ResultsIn sciatic nerves of CIDP patients, mean cross‐sectional area was significantly higher and fractional anisotropy value was significantly lower, compared to controls. In contrast, muscle proton‐density fat fraction was significantly higher in thigh muscles of patients with CIDP, compared to controls. MRI parameters showed high reproducibility at baseline and 6 months.InterpretationAdvanced MRI parameters demonstrate subclinical proximal nerve damage and intramuscular fat accumulation in CIDP. Data suggest DTI and multiecho Dixon MRI might be useful in estimating axonal damage and neurogenic muscle changes in CIDP.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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MRI biomarkers of proximal nerve injury in CIDP

Lichtenstein

Sprenger

Weiss

et al. 2017

Ann Clin Transl Neurol

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“…Studies in facioscapulohumeral muscular dystrophy (FSHD) have also shown that fat fractions derived from Dixon imaging correspond to disease‐specific scales and clinical outcome measures . Dixon imaging has similarly been used to examine skeletal muscles in the Becker and limb‐girdle muscular dystrophy populations …”

Section: Evaluating Intramuscular Fatmentioning

confidence: 99%

“…LGMD2I, limb-girdle muscular dystrophy type 2I examine skeletal muscles in the Becker and limb-girdle muscular dystrophy populations. 19,30,51 2.2.2 | T2-Weighted Imaging T2-weighted sequences generate contrasts in images on the basis of the transverse relaxation times of protons. Both fat and free water appear hyperintense on T2-weighted sequences, and investigators have used T2-weighted sequences to quantitate fat infiltration in muscle (similarly to T1-weighted images).…”

Section: Chemical Shift-based Imaging (Dixon Techniques)mentioning

confidence: 99%

Advancements in magnetic resonance imaging‐based biomarkers for muscular dystrophy

Leung

2019

Muscle and Nerve

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Recent years have seen steady progress in the identification of genetic muscle diseases as well as efforts to develop treatment for these diseases. Consequently, sensitive and objective new methods are required to identify and monitor muscle pathology. Magnetic resonance imaging offers multiple potential biomarkers of disease severity in the muscular dystrophies. This Review uses a pathology‐based approach to examine the ways in which MRI and spectroscopy have been used to study muscular dystrophies. Methods that have been used to quantitate intramuscular fat, edema, fiber orientation, metabolism, fibrosis, and vascular perfusion are examined, and this Review describes how MRI can help diagnose these conditions and improve upon existing muscle biomarkers by detecting small increments of disease‐related change. Important challenges in the implementation of imaging biomarkers, such as standardization of protocols and validating imaging measurements with respect to clinical outcomes, are also described.

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Natural history of limb girdle muscular dystrophy R9 over 6 years: searching for trial endpoints

Murphy

Morrow

Dahlqvist

et al. 2019

Ann Clin Transl Neurol

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Objective Limb girdle muscular dystrophy type R9 (LGMD R9) is an autosomal recessive muscle disease for which there is currently no causative treatment. The development of putative therapies requires sensitive outcome measures for clinical trials in this slowly progressing condition. This study extends functional assessments and MRI muscle fat fraction measurements in an LGMD R9 cohort across 6 years. Methods Twenty‐three participants with LGMD R9, previously assessed over a 1‐year period, were re‐enrolled at 6 years. Standardized functional assessments were performed including: myometry, timed tests, and spirometry testing. Quantitative MRI was used to measure fat fraction in lower limb skeletal muscle groups. Results At 6 years, all 14 muscle groups assessed demonstrated significant increases in fat fraction, compared to eight groups in the 1‐year follow‐up study. In direct contrast to the 1‐year follow‐up, the 6‐min walk test, 10‐m walk or run, timed up and go, stair ascend, stair descend and chair rise demonstrated significant decline. Among the functional tests, only FVC significantly declined over both the 1‐ and 6‐year studies. Interpretation These results further support fat fraction measurements as a primary outcome measure alongside functional assessments. The most appropriate individual muscles are the vastus lateralis, gracilis, sartorius, and gastrocnemii. Using composite groups of lower leg muscles, thigh muscles, or triceps surae, yielded high standardized response means (SRMs). Over 6 years, quantitative fat fraction assessment demonstrated higher SRM values than seen in functional tests suggesting greater responsiveness to disease progression.

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The 6-minute walk test, motor function measure and quantitative thigh muscle MRI in Becker muscular dystrophy: A cross-sectional study

Cited by 37 publications

References 33 publications

MRI biomarkers of proximal nerve injury in CIDP

MRI biomarkers of proximal nerve injury in CIDP

Advancements in magnetic resonance imaging‐based biomarkers for muscular dystrophy

Natural history of limb girdle muscular dystrophy R9 over 6 years: searching for trial endpoints

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