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Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy.

Authors :
Fischmann, Arne
Gloor, Monika
Fasler, Susanne
Haas, Tanja
Rodoni Wetzel, Rachele
Bieri, Oliver
Wetzel, Stephan
Heinimann, Karl
Scheffler, Klaus
Fischer, Dirk
Source :
Journal of Neurology. Jul2011, Vol. 258 Issue 7, p1333-1340. 8p. 2 Black and White Photographs, 2 Charts, 1 Graph.
Publication Year :
2011

Abstract

Oculopharyngeal muscular dystrophy (OPMD) is a progressive skeletal muscle dystrophy characterized by ptosis, dysphagia, and upper and lower extremity weakness. We examined eight genetically confirmed OPMD patients to detect a MRI pattern and correlate muscle involvement, with validated clinical evaluation methods. Physical assessment was performed using the Motor Function Measurement (MFM) scale. We imaged the lower extremities on a 1.5 T scanner. Fatty replacement was graded on a 4-point visual scale. We found prominent affection of the adductor and hamstring muscles in the thigh, and soleus and gastrocnemius muscles in the lower leg. The MFM assessment showed relative mild clinical impairment, mostly affecting standing and transfers, while distal motor capacity was hardly affected. We observed a high (negative) correlation between the validated clinical scores and our visual imaging scores suggesting that quantitative and more objective muscle MRI might serve as outcome measure for clinical trials in muscular dystrophies. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
03405354
Volume :
258
Issue :
7
Database :
Academic Search Index
Journal :
Journal of Neurology
Publication Type :
Academic Journal
Accession number :
62518522
Full Text :
https://doi.org/10.1007/s00415-011-5937-9