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Ann Clin Neurophysiol > Volume 21(2); 2019 > Article
ORIGINAL ARTICLE
Ann Clin Neurophysiol. 2019; 21(2): 79-86.
Published online July 31, 2019.
doi: https://doi.org/10.14253/acn.2019.21.2.79
Pattern analysis of lower limb magnetic resonance images in Korean patients with distal myopathy
Hyung Jun Park1,2  , Ha Young Shin2  , Seung Min Kim2  , Kee Duk Park3  , and Young-Chul Choi2 
1Department of Neurology, Gangneung Asan Hospital, University of Ulsan College of Medicine, Gangneung, Korea
2Department of Neurology, Yonsei University College of Medicine, Seoul, Korea
3Department of Neurology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul, Korea
Corresponding Author: Young-Chul Choi ,Tel: +82-2-2019-3323, Fax: +82-2-3462-5904, Email: ycchoi@yuhs.ac
Received June 5, 2018   Revised: July 10, 2018    Accepted July 18, 2018
ABSTRACT
Background: Magnetic resonance (MR) images are useful for diagnosing myopathy. The purpose of this study was to determine the usefulness of lower-limb MR images in Korean patients with distal myopathy.
Methods: We reviewed medical records in the myopathy database from January 2002 to October 2016. We selected 21 patients from 91 unrelated families with distal myopathy: four with GNE myopathy, 11 with dysferlinopathy, and six with ADSSL1 myopathy.
Results: Ten (48%) of the 21 patients were men. The ages of the participants at symptom onset and imaging were 19.2 ± 9.5 and 30.4 ± 9.0 years (mean ± standard deviation), respectively. Their grade on the modified Gardner-Medwin and Walton grade was 3.3 ± 1.7. The strength grade of the knee extensors was not correlated with the Mercuri scale for the quadriceps (r = –0.247, p = 0.115). However, the Medical Research Council grades of the knee flexors, ankle dorsiflexors, and ankle plantar flexors were significantly correlated with the Mercuri scale ratings of the knee flexors (r = –0.497, p = 0.001), tibialis anterior (r = –0.727, p < 0.001), and ankle plantar flexors (r = –0.620, p < 0.001), respectively. T1-weighted MR images showed characteristic fatty replacement patterns that were consistent with the causative genes. Unsupervised hierarchical clustering of the Mercuri scale showed that the main factors contributing to the dichotomy were the causative gene and the clinical severity.
Conclusions: This study is the first to reveal the usefulness of lower-limb MR images in the differential diagnosis of distal myopathy in Korea.
Key words: Distal myopathies; Magnetic resonance imaging; DYSF; GNE; ADSSL1
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