Abstract
Background and Objectives: The aim of this study was to evaluate longitudinal changes using both upper limb muscle Magnetic Resonance Imaging (MRI) at shoulder, arm and forearm levels and Performance of upper limb (PUL) in ambulant and non-ambulant Duchenne Muscular Dystrophy (DMD) patients. We also wished to define whether baseline muscle MRI could help to predict functional changes after one year. Materials and Methods: Twenty-seven patients had both baseline and 12month muscle MRI and PUL assessments one year later. Results: Ten were ambulant (age range 5–16 years), and 17 non ambulant (age range 10–30 years). Increased abnormalities equal or more than 1.5 point on muscle MRI at follow up were found on all domains: at shoulder level 12/27 patients (44%), at arm level 4/27 (15%) and at forearm level 6/27 (22%). Lower follow up PUL score were found in 8/27 patients (30%) at shoulder level, in 9/27 patients (33%) at mid-level whereas no functional changes were found at distal level. There was no constant association between baseline MRI scores and follow up PUL scores at arm and forearm levels but at shoulder level patients with moderate impairment on the baseline MRI scores between 16 and 34 had the highest risk of decreased function on PUL over a year. Conclusions: Our results confirmed that the integrated use of functional scales and imaging can help to monitor functional and MRI changes over time.
Highlights
In the last few years, the development of new outcome measures has allowed a better definition of the natural history of Duchenne Muscular Dystrophy (DMD), a progressive, X-linked neuromuscular disorder caused by mutation in the dystrophin gene, affecting one in 3600–5000 live male births
While in the past the severity of muscle involvement was mainly assessed by muscle biopsy, over the last two decades there has been increasing attention to the use of noninvasive techniques, such as Magnetic Resonance Imaging (MRI) and spectroscopy (MRS)
Most MRI studies have focused on lower limbs and less has been reported on possible changes in upper limb involvement
Summary
Most MRI studies have focused on lower limbs and less has been reported on possible changes in upper limb involvement. As suggested in our recent cross-sectional study investigating all upper limb domains (proximal, mid, distal) in combination with functional scale including Performance of Upper Limb (PUL) test [26]. The aim of this study was to evaluate longitudinal changes using both upper limb muscle Magnetic Resonance Imaging (MRI) at shoulder, arm and forearm levels and Performance of upper limb (PUL) in ambulant and non-ambulant Duchenne Muscular. We wished to define whether baseline muscle MRI could help to predict functional changes after one year. Materials and Methods: Twenty-seven patients had both baseline and 12month muscle MRI and PUL assessments one year later.
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