Relationships of thigh muscle contractile and non-contractile tissue with function, strength, and age in boys with Duchenne muscular dystrophy

Akima, Hiroshi; Lott, Donovan J.; Senesac, Claudia; Deol, Jasjit; Germain, Sean; Arpan, Ishu; Bendixen, Roxanna; Sweeney, H. Lee; Walter, Glenn A.; Vandenborne, Krista

doi:10.1016/j.nmd.2011.06.750

Cited by 86 publications

(130 citation statements)

References 36 publications

Supporting

Mentioning

125

Contrasting

Unclassified

Order By: Relevance

“…Fat infiltration correlated with clinical assessments such as timed walking/running or rising from supine tests, manual muscle testing, MFM, or Brooke scales. 8,9,[11][12][13]15,19,20,32,33 %F in lower limb has been reported previously to be predictive of ambulation loss and linearly correlated with MFM 9 and strength, 19 whereas metabolic factors did not correlate linearly. We reproduced these findings in upper limbs of nonambulatory patients.…”

Section: Resultsmentioning

confidence: 71%

“…Despite pioneering NMRS work in forearm muscles of patients with DMD, 7 NMR data have since been primarily acquired in pelvic muscles or lower limbs in ambulant patients. [8][9][10][11][12][13][14][15][16][17][18][19][20] We recently reported the feasibility of using NMR for assessing the upper limb in nonambulatory patients. 21 Our main objective in the present study was to establish the baseline values for NMR and functional variables and at a 1-year follow-up time point period for ambulatory and nonambulatory patients with DMD.…”

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy

et al. 2016

View full text Add to dashboard Cite

Objective: To explore the value of nuclear magnetic resonance (NMR) and functional assessments for follow-up of ambulatory and nonambulatory patients with Duchenne muscular dystrophy (DMD).Methods: Twenty-five 53-skippable patients with DMD were included in this study; 15 were nonambulatory at baseline. All patients underwent clinical and functional assessments every 6 months using the Motor Function Measure (MFM), hand grip and key pinch strength, MoviPlate, and NMR spectroscopy and imaging studies.Results: Upper limb distal strength decreased in nonambulatory patients over the period of 1 year; ambulatory patients showed improvement during the same period. The same applied for several NMRS indices, such as phosphocreatine/adenosine triphosphate, which decreased in older patients but increased in younger ambulatory patients. Fat infiltration in the upper limbs increased linearly with age. Almost all NMR and functional assessment results correlated. Conclusions:Our results underscore complementarity of functional and NMR assessments in patients with DMD. Sensitivity to change of various indices may differ according to disease stage. MFM 5 Motor Function Measure; NMR 5 nuclear magnetic resonance; NMRI 5 nuclear magnetic resonance imaging; NMRS 5 nuclear magnetic resonance spectroscopy; PCr 5 phosphocreatine; Pi 5 inorganic phosphate; Pia 5 cytosolic inorganic phosphate; Pib 5 anomalous alkaline pool present in dystrophic muscle; PDE 5 phosphodiester; PME 5 phosphomonoester; TR 5 repetition time.

show abstract

Section: Resultsmentioning

confidence: 71%

Section: Resultsmentioning

confidence: 99%

Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy

et al. 2016

View full text Add to dashboard Cite

show abstract

“…Of the muscle groups studied, the Gra muscle was the only exception. Gra is known to be one of the most preserved muscles in DMD 20,22,30 and hence might not reveal treatment response at such a young age. Likewise, MRS T2 was lower in the Sol and VL muscles of corticosteroid-treated boys.…”

Section: Impact Of Initiation Of Corticosteroid Therapy In Boysmentioning

confidence: 99%

Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS

et al. 2014

View full text Add to dashboard Cite

Objective: To evaluate the effects of corticosteroids on the lower extremity muscles in boys with Duchenne muscular dystrophy (DMD) using MRI and magnetic resonance spectroscopy (MRS).Methods: Transverse relaxation time (T2) and fat fraction were measured by MRI/MRS in lower extremity muscles of 15 boys with DMD (age 5.0-6.9 years) taking corticosteroids and 15 corticosteroid-naive boys. Subsequently, fat fraction was measured in a subset of these boys at 1 year. Finally, MRI/MRS data were collected from 16 corticosteroid-naive boys with DMD (age 5-8.9 years) at baseline, 3 months, and 6 months. Five boys were treated with corticosteroids after baseline and the remaining 11 served as corticosteroid-naive controls. Results:Cross-sectional comparisons demonstrated lower muscle T2 and less intramuscular (IM) fat deposition in boys with DMD on corticosteroids, suggesting reduced inflammation/damage and fat infiltration with treatment. Boys on corticosteroids demonstrated less increase in IM fat infiltration at 1 year. Finally, T2 by MRI/MRS detected effects of corticosteroids on leg muscles as early as 3 months after drug initiation.Conclusions: These results demonstrate the ability of MRI/MRS to detect therapeutic effects of corticosteroids in reducing inflammatory processes in skeletal muscles of boys with DMD. Our work highlights the potential of MRI/MRS as a biomarker in evaluating therapeutic interventions in DMD. Duchenne muscular dystrophy (DMD) is a devastating form of muscular dystrophy caused by the absence of dystrophin, making muscle cell membranes fragile and susceptible to mechanical damage.1,2 Currently, there is no cure for the disease. Corticosteroids have been reported to slow disease progression in DMD.3-7 However, the mechanism by which corticosteroids preserve muscle function in DMD is not fully understood.Among several proposed mechanisms, corticosteroids are thought to reduce inflammation in dystrophic muscles. 8,9 MRI, in particular T2-weighted MRI, is sensitive to alterations in muscle chemistry and structure induced by processes like damage/inflammation and fat infiltration, [10][11][12][13][14][15][16][17] and therefore may have the potential to detect the effects of corticosteroid treatment on dystrophic muscles. Magnetic resonance spectroscopy (MRS) allows quantification of chemical compounds and can separate lipid and water components, allowing a more targeted investigation of skeletal muscles in DMD. 18-21The overall goal of this study was to examine the ability of MRI/MRS to detect the effects of corticosteroids on skeletal muscles in boys with DMD. The specific aims of the study were to (1) perform a cross-sectional comparison between the lower extremity muscles of 5-to 6.9-year-old

show abstract

“…Bone was eliminated, and a region of interest was manually defined at the level of the deep fascia for the same 5 slices analyzed for muscle volumes. Separation of contractile and noncontractile tissue was achieved by creating a voxel intensity histogram for each drawn region of interest and implementing the maximum entropy method available within MIPAV (59). Data were exported as volumes-per-voxel intensity and separated above (adipose tissue) and below (contractile tissue) the determined threshold value.…”

Section: Figurementioning

confidence: 99%

Essential amino acid supplementation in patients following total knee arthroplasty

Dreyer¹,

Strycker²,

Senesac³

et al. 2013

J. Clin. Invest.

View full text Add to dashboard Cite

Background. By the year 2030, 3.48 million older U.S. adults are projected to undergo total knee arthroplasty (TKA). Following this surgery, considerable muscle atrophy occurs, resulting in decreased strength and impaired functional mobility. Essential amino acids (EAAs) have been shown to attenuate muscle loss during periods of reduced activity and may be beneficial for TKA patients.Methods. We used a double-blind, placebo-controlled, randomized clinical trial with 28 older adults undergoing TKA. Patients were randomized to ingest either 20 g of EAAs (n = 16) or placebo (n = 12) twice daily between meals for 1 week before and 2 weeks after TKA. At baseline, 2 weeks, and 6 weeks after TKA, an MRI was performed to determine mid-thigh muscle and adipose tissue volume. Muscle strength and functional mobility were also measured at these times.Results. TKA patients receiving placebo exhibited greater quadriceps muscle atrophy, with a -14.3 ± 3.6% change from baseline to 2 weeks after surgery compared with -3.4 ± 3.1% for the EAA group (F = 5.16, P = 0.036) and a -18.4 ± 2.3% change from baseline to 6 weeks after surgery for placebo versus -6.2 ± 2.2% for the EAA group (F = 14.14, P = 0.001). EAAs also attenuated atrophy in the nonoperated quadriceps and in the hamstring and adductor muscles of both extremities. The EAA group performed better at 2 and 6 weeks after surgery on functional mobility tests (all P < 0.05). Change in quadriceps muscle atrophy was significantly associated with change in functional mobility (F = 5.78, P = 0.021).Conclusion. EAA treatment attenuated muscle atrophy and accelerated the return of functional mobility in older adults following TKA.Trial registration. Clinicaltrials.gov NCT00760383.

show abstract

Relationships of thigh muscle contractile and non-contractile tissue with function, strength, and age in boys with Duchenne muscular dystrophy

Cited by 86 publications

References 36 publications

Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy

Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy

Examination of effects of corticosteroids on skeletal muscles of boys with DMD using MRI and MRS

Essential amino acid supplementation in patients following total knee arthroplasty

Contact Info

Product

Resources

About