Duchenne muscular dystrophy: MR grading system with functional correlation.

Liu, Gin-Chung; Jong, Yuh‐Jyh; Cw, Chiang; Jaw, Twei-Shiun

doi:10.1148/radiology.186.2.8421754

Cited by 112 publications

(85 citation statements)

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“…Asymmetric individual muscle involvement has been described in the lower limb muscles of patients with DMD in a single previous study, a finding due to the high resolution of muscle MRI. 18 We did not observe any obvious asymmetric involvement in the transverse sections of the below the knees muscle groups in our cohort, although this is a phenomenon that can be found in female manifesting carriers of DMD. 19 We have not found any patient with absent EDB muscles, which is a normal but very rare variation in the general population.…”

contrasting

confidence: 52%

“…Our data complement those of a previous study demonstrating a good correlation between MRI grading of the lower limb muscles (thighs and calves) and the patients' clinical function as well as their disease duration. 18 Nevertheless, individual patients showed unexpected relative preservation (grade 2 changes) on MRI grading despite advanced age (mean age 16 years, mean 4 years since loss of ambulation). In 6 patients there was an asymmetric MRI involvement with regard to muscle bulk and structural changes, with 1 of the 2 EDB muscles being slightly better preserved (mostly grades 2a/2b).…”

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confidence: 96%

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Muscle histology vs MRI in Duchenne muscular dystrophy

Kinali¹,

Arechavala‐Gomeza²,

Çırak³

et al. 2011

Neurology

139

127

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Objective: There are currently no effective treatments to halt the muscle breakdown in Duchenne muscular dystrophy (DMD), although genetic-based clinical trials are being piloted. Most of these trials have as an endpoint the restoration of dystrophin in muscle fibers, hence requiring sufficiently well-preserved muscle of recruited patients. The choice of the muscles to be studied and the role of noninvasive methods to assess muscle preservation therefore require further evaluation. Methods:We studied the degree of muscle involvement in the lower leg muscles of 34 patients with DMD Ͼ8 years, using muscle MRI. In a subgroup of 15 patients we correlated the muscle MRI findings with the histology of open extensor digitorum brevis (EDB) muscle biopsies. Muscle MRI involvement was assigned using a scale 0-4 (normal-severe). Results:In all patients we documented a gradient of involvement of the lower leg muscles: the posterior compartment (gastrocnemius Ͼ soleus) was most severely affected; the anterior compartment (tibialis anterior/posterior, popliteus, extensor digitorum longus) least affected. Muscle MRI showed EDB involvement that correlated with the patient's age (p ϭ 0.055). We show a correlation between the MRI and EDB histopathologic changes, with MRI 3-4 grades associated with a more severe fibro-adipose tissue replacement. The EDB was sufficiently preserved for bulk and signal intensity in 18/22 wheelchair users aged 10-16.6 years. Conclusion:This study provides a detailed correlation between muscle histology and MRI changes in DMD and demonstrates the value of this imaging technique as a reliable tool for the selection of muscles in patients recruited into clinical trials. Neurology ® 2011;76:346-353 GLOSSARY AO ϭ antisense oligonucleotide; DMD ϭ Duchenne muscular dystrophy; EDB ϭ extensor digitorum brevis; KAFO ϭ knee-ankle-foot orthosis; NSA ϭ number of signal averages; TE ϭ echo time; TR ϭ repetition time.The progressive loss of muscle fibers and their replacement by fat and connective tissue is part of the disease course of DMD.1 Despite several exhaustive natural history studies on the clinical course of DMD, the progression of pathology in individual muscle groups is poorly defined. 2Information on muscle pathology in DMD is limited to the muscles that are biopsied at the time of diagnosis (often quadriceps femoris), 2 the mean age at diagnosis being ϳ4.5 years. 3,4 There are now early clinical trials using novel genetic-based techniques in boys with DMD with the aim to restore dystrophin expression. These include the drug PTC124, 5 which allows

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contrasting

confidence: 52%

mentioning

confidence: 96%

Muscle histology vs MRI in Duchenne muscular dystrophy

Kinali¹,

Arechavala‐Gomeza²,

Çırak³

et al. 2011

Neurology

139

127

View full text Add to dashboard Cite

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“…Thus, these proportions of long muscle fibers may not be representative of the entire muscle. Sartorius and gracilis muscles are selectively spared in a number of human muscle diseases (Lamminen, 1990;Liu et al, 1993). This may reflect their possession of a proportion of muscle fibers that terminate intrafascicularly and hence are less susceptible to injury during eccentric contraction (Childers et al, 2002) than fibers that extend the full length of the muscle.…”

Section: Discussionmentioning

confidence: 99%

Muscle Fiber and Motor Unit Behavior in the Longest Human Skeletal Muscle

Harris¹,

Duxson²,

Butler³

et al. 2005

J. Neurosci.

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The sartorius muscle is the longest muscle in the human body. It is strap-like, up to 600 mm in length, and contains five to seven neurovascular compartments, each with a neuromuscular endplate zone. Some of its fibers terminate intrafascicularly, whereas others may run the full length of the muscle. To assess the location and timing of activation within motor units of this long muscle, we recorded electromyographic potentials from multiple intramuscular electrodes along sartorius muscle during steady voluntary contraction and analyzed their activity with spike-triggered averaging from a needle electrode inserted near the proximal end of the muscle. Approximately 30% of sartorius motor units included muscle fibers that ran the full length of the muscle, conducting action potentials at 3.9 Ϯ 0.1 m/s. Most motor units were innervated within a single muscle endplate zone that was not necessarily near the midpoint of the fiber. As a consequence, action potentials reached the distal end of a unit as late as 100 ms after initiation at an endplate zone. Thus, contractile activity is not synchronized along the length of single sartorius fibers. We postulate that lateral transmission of force from fiber to endomysium and a wide distribution of motor unit endplates along the muscle are critical for the efficient transmission of force from sarcomere to tendon and for the prevention of muscle injury caused by overextension of inactive regions of muscle fibers.

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“…Liu, Y.J. Jong et al [10]. Результаты МРТ говорят об относительной сохранности m. gracilis, sartorius, semitendinous и semimembranous.…”

Section: обс у ж дениеunclassified

Correlation analysis of T2-weighted images and Medical Research Council scale in corticosteroid-naive patients with Duchenne muscular dystrophy

et al. 2017

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Because of the necessity of objective instrumental methods for assessing the state of skeletal musculature in patients with Duchenne muscular dystrophy, the aim of our work was to evaluate the correlation between manual methods of assessment of the muscle strength and the results of magnetic resonance imaging (MRI) of the skeletal muscles of lower limbs. We have examined 15 corticosteroid-naive patients with Duchenne muscular dystrophy. Patients were divided into 2 groups: ambulant patients (average age 8.1 years) and non-ambulant patients (mean age 12.7 years). Muscle strength of lower extremities of all patients has been evaluated by Medical Research Council scale and MRI of skeletal muscles of the pelvic girdle, thighs and lower legs has been performed. The following results have been obtained: ambulant patients have been characterized by a high correlation of MRC scale and MRI of the lower extremities in the evaluation of the pelvic girdle and thighs muscles and the total score (pelvic girdle, thighs and lower legs) and salient correlation in the evaluation of the lower legs muscles. Non-ambulant patients have been characterized by moderate correlation in the evaluation of pelvic girdle and thighs muscles, as well as the total score of lower limbs. There was no correlation between MRC and MRI results in the muscles of the lower legs. Magnetic resonance tomography is able to reliably estimate the degree of fatty infiltration in Duchenne muscular dystrophy and is the method of choice in the diagnosis and evaluation of the severity of this disease.

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Duchenne muscular dystrophy: MR grading system with functional correlation.

Cited by 112 publications

References 0 publications

Muscle histology vs MRI in Duchenne muscular dystrophy

Muscle histology vs MRI in Duchenne muscular dystrophy

Muscle Fiber and Motor Unit Behavior in the Longest Human Skeletal Muscle

Correlation analysis of T2-weighted images and Medical Research Council scale in corticosteroid-naive patients with Duchenne muscular dystrophy

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