caa a22 4500 445808179 CHVBK 20180317145203.0 cr unu---uuuuu 170323e20110701xx s 000 0 eng 10.1007/s00415-011-5937-9 doi (NATIONALLICENCE)springer-10.1007/s00415-011-5937-9 Muscular involvement assessed by MRI correlates to motor function measurement values in oculopharyngeal muscular dystrophy [Elektronische Daten] [Arne Fischmann, Monika Gloor, Susanne Fasler, Tanja Haas, Rachele Rodoni Wetzel, Oliver Bieri, Stephan Wetzel, Karl Heinimann, Klaus Scheffler, Dirk Fischer] 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 1.5T 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. Springer-Verlag, 2011 MRI nationallicence Motor function measurement nationallicence Outcome measure nationallicence Muscle nationallicence Oculopharyngeal muscular dystrophy nationallicence OPMD nationallicence Fischmann Arne Department of Neuroradiology, Institute of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland aut Gloor Monika Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut Fasler Susanne Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut Haas Tanja Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut Rodoni Wetzel Rachele Physiotherapy Neurology, University of Basel Hospital, Basel, Switzerland aut Bieri Oliver Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut Wetzel Stephan Department of Neuroradiology, Swiss Neuro Institute, Hirslanden Klinik Zurich, Zurich, Switzerland aut Heinimann Karl Department of Medical Genetics, University of Basel Children's Hospital, Basel, Switzerland aut Scheffler Klaus Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut Fischer Dirk Department of Neuropaediatrics, University Children's Hospital, Basel, Switzerland aut Journal of Neurology Springer-Verlag 258/7(2011-07-01), 1333-1340 0340-5354 258:7<1333 2011 258 415 https://doi.org/10.1007/s00415-011-5937-9 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-011-5937-9 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Fischmann Arne Department of Neuroradiology, Institute of Radiology, University of Basel Hospital, Petersgraben 4, 4031, Basel, Switzerland aut NATIONALLICENCE 700 1- Gloor Monika Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Fasler Susanne Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Haas Tanja Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Rodoni Wetzel Rachele Physiotherapy Neurology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Bieri Oliver Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Wetzel Stephan Department of Neuroradiology, Swiss Neuro Institute, Hirslanden Klinik Zurich, Zurich, Switzerland aut NATIONALLICENCE 700 1- Heinimann Karl Department of Medical Genetics, University of Basel Children's Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Scheffler Klaus Department of Radiological Physics, Institute of Radiology, University of Basel Hospital, Basel, Switzerland aut NATIONALLICENCE 700 1- Fischer Dirk Department of Neuropaediatrics, University Children's Hospital, Basel, Switzerland aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/7(2011-07-01), 1333-1340 0340-5354 258:7<1333 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer