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Clinical Trial
. 2016 Mar 15;86(11):1022-30.
doi: 10.1212/WNL.0000000000002464. Epub 2016 Feb 17.

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

Jean-Yves Hogrel  1 , Claire Wary  2 , Amélie Moraux  2 , Noura Azzabou  2 , Valérie Decostre  2 , Gwenn Ollivier  2 , Aurélie Canal  2 , Charlotte Lilien  2 , Isabelle Ledoux  2 , Mélanie Annoussamy  2 , Nacera Reguiba  2 , Teresa Gidaro  2 , Anne Gaelle Le Moing  2 , Ruxandra Cardas  2 , Thomas Voit  2 , Pierre G Carlier  2 , Laurent Servais  2
Affiliations
Clinical Trial

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

Jean-Yves Hogrel et al. Neurology. .

Abstract

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.

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Figures

Figure 1
Figure 1. Correlations between various functional and nuclear magnetic resonance variables and age
Variables plotted against age: grip strength (A), pinch strength (B), MoviPlate score (C), dimension 3 of the Muscle Function Measure (MFM-D3) (D), % fat (E), and phosphocreatine/adenosine triphosphate (F). Spearman correlations. Ambulatory patients represented in blue; nonambulatory patients in red. *The patient with more preserved strength, considered an outlier.
Figure 2
Figure 2. Correlations between grip strength and nuclear magnetic resonance variables
Variables plotted against grip strength: T2 (A), % fat (B), anomalous alkaline pool present in dystrophic muscle (Pib)/inorganic phosphate (Pi) (C), cytosolic inorganic phosphate (Pia)/phosphocreatine (PCr) (D), phosphodiester (PDE)/adenosine triphosphate (ATP) (E), and PCr/ATP (F). Spearman correlations. Ambulatory patients represented in blue; nonambulatory patients in red. *The patient with more preserved strength, considered an outlier.
See this image and copyright information in PMC

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