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Ad dress for Cor res pon den ce/Ya z›fl ma Ad re si: Hacer Durmuş MD, İstanbul Üniversity İstanbul Faculty of Medicine, Department of Nöurology, İstanbul, Turkey Gsm: +90 507 947 74 27 E-mail: durmushacer@yahoo.com Re cei ved/Ge lifl Ta ri hi: 20.10.2014 Ac cep ted/Ka bul Ta ri hi: 06.11.2014 Objective: Oculopharyngodistal myopathy (OPDM) has been reported as a rare, adult-onset hereditary muscle disease. Patients show progressive oculopharyngeal and distal limb muscle involvement. As the genetic defect underlying OPDM is not known yet, the diagnosis currently rests upon clinical and histopathological features. This study was aimed at investigating patterns of muscle alterations of OPDM patients by MRI and to search for possible clues to make differential diagnosis by using a non-invasive method. Ma te ri als and Met hods: Facial, upper and lower extremity muscles of 10 patients with OPDM, followed by the Neuromuscular Unit, Department of Neurology, Istanbul Faculty of Medicine, who had undergone detailed evaluation with manual muscle testing and who had different disease severity were evaluated with MRI using conventional T1 and T2 weighted axial images. The degree of muscle involvement on MRI was evaluated according to a modified 5-point scale in extremities and 6-point scale in facial muscles. Re sults: The mean age of onset was 20.1±8.2 years (range 7-39 years) and the mean disease duration was 14.5±12.4 years (range 2-41 years). Seven patients showed dominantly distal, one patient dominantly proximal weakness and two patients had no weakness. The patients without weakness had normal imaging, but facial muscle MRI from one of them revealed mild involvement. Zygomatic and nasal muscles were the most severely and earliest involved muscles in face. MRI of all patients with muscle weakness showed a consistent selective extremity muscle involvement pattern. Distal extremity muscles were more affected than proximal muscles. Earliest and most sever changes were found in semimembranosus, biceps femoris and medial head of gastrocnemius, soleus. Interestingly, sartorius, gracilis and semitendinous muscles and the lateral head of gastrocnemius were well-preserved in OPDM. Conclusion: Muscle MRI by showing selective involvement of exteremity muscle may be a non-invasive tool in the differential diagnosis of OPDM. (Turkish Journal of Neurology 2014; 20:121-125)

Facial and Skeletal Muscle Magnetic Resonance Imaging In Oculopharyngodistal Myopathy