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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.
GLOSSARY
- %F=
- fat percentage;
- 6MWT=
- 6-Minute Walk Test;
- ATP=
- adenosine triphosphate;
- DMD=
- Duchenne muscular dystrophy;
- MFM=
- Motor Function Measure;
- NMR=
- nuclear magnetic resonance;
- NMRI=
- nuclear magnetic resonance imaging;
- NMRS=
- nuclear magnetic resonance spectroscopy;
- PCr=
- phosphocreatine;
- Pi=
- inorganic phosphate;
- Pia=
- cytosolic inorganic phosphate;
- Pib=
- anomalous alkaline pool present in dystrophic muscle;
- PDE=
- phosphodiester;
- PME=
- phosphomonoester;
- TR=
- repetition time
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work as first authors.
↵‡ These authors contributed equally to this work as last authors.
- Received April 13, 2015.
- Accepted in final form December 3, 2015.
- © 2016 American Academy of Neurology
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