Analysis of fatty infiltration and inflammation of the pelvic and thigh muscles in boys with Duchenne muscular dystrophy (DMD): Grading of disease involvement on MR imaging and correlation with clinical assessments
BACKGROUND: Prior reports focus primarily on muscle fatty infiltration in Duchenne muscular dystrophy (DMD). However, the significance of muscle edema is uncertain. OBJECTIVE: To evaluate the frequency and degree of muscle fat and edema, and correlate these with clinical function. MATERIALS AND METHODS: Forty-two boys (ages 5-19 years) with DMD underwent pelvic MRI. Axial T1- and fat-suppressed T2-weighted images were evaluated to grade muscle fatty infiltration (0-4) and edema (0-3), respectively. Degree and frequency of disease involvement were compared to clinical evaluations. RESULTS: Gluteus maximus had the greatest mean fatty infiltration score, followed by adductor magnus and gluteus medius muscles, and had the most frequent and greatest degree of fatty infiltration. Gluteus maximus also had the greatest mean edema score, followed by vastus lateralis and gluteus medius muscles. These muscles had the most frequent edema, although the greatest degree of edema was seen in other muscles. There was correlation between cumulative scores of fatty infiltration and all clinical evaluations (P < 0.05). CONCLUSION: In DMD, the muscles with the most frequent fatty infiltration had the greatest degree of fatty infiltration and correlated with patient function. However, the muscles with the most frequent edema were different from those with the greatest degree of edema. Thus, edema may not predict patient functional status.
"The impressive objective finding was MRI-msk with no increase in the fatty infiltration (Figure 2A, 2B). Analysis of serial MRI scans of children with DMD suggests a 5% increase of fatty infiltration every year . No significant increase in the fatty infiltration in 36 months suggests altered disease progression. "
[Show abstract][Hide abstract] ABSTRACT: Patient: Male, 9 Final Diagnosis: Duchenne muscular dystrophy Symptoms: Hyporeflexia • hypotonia • weaknes of lower limbs Medication: - Clinical Procedure: - Specialty: Neurology.
Duchenne muscular dystrophy (DMD) is a fatal, genetic, progressive, degenerating muscle disorder. Current treatment options are palliative. Newer options of cellular therapy promise to alter the disease process. Preclinical studies have successfully tested myogenic, neurogenic potential and dystrophin expression of bone marrow mononuclear cells.
We treated a 9-year-old boy suffering from DMD with serial autologous bone marrow mononuclear cell transplantations followed by multidisciplinary rehabilitation. Brooke-Vignos score was 10 and he was wheelchair-bound. Over 36 months, gradual progressive improvement was noticed in muscle strength, ambulation with assistive devices, fine motor movements, Brooke-Vignos score, and functional independence measure score. Nine months after the transplantation, electromyography findings showed development of new normal motor unit potentials of the vastus medialis muscle.
Magnetic resonance imaging scan of musculoskeletal systems showed no increase in fatty infiltration. This case report provides early investigative findings or the restorative effects of cellular therapy in DMD.
American Journal of Case Reports 03/2014; 15:128-34. DOI:10.12659/AJCR.890078
[Show abstract][Hide abstract] ABSTRACT: The purpose of this study was to use proton magnetic resonance spectroscopy to assess intramuscular lipid and metabolites of lower leg muscles in boys with Duchenne muscular dystrophy (DMD) and determine its relationship with strength and functional ability. Spectroscopic measurements were obtained from four muscles of the lower leg in 25 boys with DMD (9.2±3.1 years) and 10 healthy boys (10.2±2.6 years). Lipid fractions and metabolite concentrations were also determined. Muscle strength, a timed functional test, and the Modified Brooke Lower Extremity Functional Scale were also determined. Lipid fractions were higher (p<0.01) for the DMD group than healthy subjects for all muscles, and lipid fraction was found to be greater in the older DMD boys. The peroneal muscle demonstrated a significant difference in lipid fraction in all DMD age groups. Lipid fractions in all muscles correlated with functional measures (r=0.52-0.70, p<0.001), with smaller inverse correlations with the strength measure (r=-0.36 - -0.56, p<0.05). These findings provide quantifiable information regarding intramuscular lipid and metabolite levels of different muscles across various age groups in boys with DMD and may be used in determining the effect of interventions in future clinical trials.
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