Fulltext

Quantitative MRI and loss of free ambulation in Duchenne muscular dystrophy

Fischmann, Arne ; Hafner, Patricia ; Gloor, Monika ; Schmid, Maurice ; Klein, Andrea ; Pohlman, Urs ; Waltz, Tanja ; Gonzalez, Rocio ; Haas, Tanja ; Bieri, Oliver ; Fischer, Dirk

In: Journal of Neurology, 2013, vol. 260, no. 4, p. 969-974

Ajouter à la liste personnelle
    Titre
    • Quantitative MRI and loss of free ambulation in Duchenne muscular dystrophy
    Auteur
    • Fischmann, Arne. Division of Diagnostic and Interventional Neuroradiology, Department of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    • Hafner, Patricia. Department of Neurology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    • Gloor, Monika. Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    • Schmid, Maurice. University of Basel Childrens Hospital, Spitalstrasse 33, 4056, Basel, Switzerland
    • Klein, Andrea. Department of Paediatric Neurology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zürich, Switzerland
    • Pohlman, Urs. University of Basel Childrens Hospital, Spitalstrasse 33, 4056, Basel, Switzerland
    • Waltz, Tanja. University of Basel Childrens Hospital, Spitalstrasse 33, 4056, Basel, Switzerland
    • Gonzalez, Rocio. University of Basel Childrens Hospital, Spitalstrasse 33, 4056, Basel, Switzerland
    • Haas, Tanja. Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    • Bieri, Oliver. Division of Radiological Physics, Department of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    • Fischer, Dirk. Department of Neurology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland
    Type de document
    • Postprint
    Identifiant OAI-PMH
    • oai:doc.rero.ch:314794
    Summary
    The purpose of this ethics approved trial was to correlate quantitative MRI with functional abilities in both ambulant and non-ambulant Duchenne muscular dystrophy (DMD). Twenty patients with genetically confirmed DMD were recruited. Physical assessment was performed using the motor function measurement (MFM) scale. Axial 3T MRI scans of the thighs were acquired using T1-weighted in- and opposed-phase images (TR=20ms, TE1=2.45ms, TE2=3.68ms, flip angle=15°) to calculate the relative fat fraction according to the two-point Dixon method in the knee extensors, flexors, and adductor muscles. The average MFM was 65.3% and correlated negatively to age (r 2=0.60). Overall mean fat fraction correlated positively to age (r 2=0.51-0.64). An average of 5% increase in mean fat fraction per year was calculated. Mean fat fraction of the quadriceps showed a high negative correlation (r 2=0.93) to the D1 (standing position and transfers) component of the MFM. A cutoff for mean fat fraction of 50% predicted loss of ambulation with a sensitivity of 100% and a specificity of 91%. Therefore, quantitative muscle MRI seems to be a promising endpoint for short clinical trials evaluating the effect of newer treatments on the time of loss of ambulation in DMD