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KMID : 0860920140160020064
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Journal of the Korean Association EMG-Electrodiagnostic Medicine
2014 Volume.16 No. 2 p.64 ~ p.69
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Clinical Characteristics of the Korean Patients with Adult Onset and Late Onset Myotonic Dystrophy Type 1
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Kim Hyun-Jeong
Kim Dae-Hyun
Park Yoon-Ghil
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Abstract
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Objectives: To investigate the clinical characteristics and complications of Korean patients with myotonic dystrophy type 1 (DM1).
Method: The medical records of 65 DM1 patients were reviewed retrospectively. We assessed the relationship between the age of onset, muscular impairment rating scale (MIRS) and complications of the DM1 with the number of cytosine-thymine-guanine (CTG) repeats.
Results: The mean age of onset was 30.5 ± 10.3 years and the mean disease duration until diagnosis was 6.1 ± 6.0 years. The age of onset was inversely correlated to the number of CTG repeats (P = 0.034). For the systemic complications, 20 of 55 patients (36.4%) had arrhythmia, 18 of 49 patients (36.7%) needed ventilator support, 4 of 52 (7.7%) had diabetes mellitus and 19 of 35 (54.3%) had cataracts. The difference between the number of CTG repeats of patients with these complications and that of patients without complications was not significant.
Conclusion: Regular follow-ups of electrocardiogram, pulmonary function test, blood sugar test and ocular examination is recommended for the patients with DM1.
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KEYWORD
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myotonic dystrophy, complication, CTG repeats
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