Natural history of cardiac involvement in myotonic dystrophy: Correlation with CTG repeats

被引:35
作者
Antonini, G
Giubilei, F
Mammarella, A
Amicucci, P
Fiorelli, M
Gragnani, F
Morino, S
Ceschin, V
Fragola, PV
Gennarelli, M
机构
[1] Univ Rome La Sapienza, Dept Neurol Sci, I-00185 Rome, Italy
[2] Univ Rome La Sapienza, Dept Med Therapy, I-00185 Rome, Italy
[3] Univ Roma Tor Vergata, Dept Biopathol & Diagnost Imaging, Rome, Italy
关键词
D O I
10.1212/WNL.55.8.1207
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The authors prospectively studied the natural course of cardiac involvement and its relationship to cytosine-thymine-guanine (CTG) expansion in 50 patients with myotonic dystrophy who were submitted to periodic cardiovascular EKG and EKG-Holter monitoring during a median follow-up of 56 months. Nineteen patients (38%) developed major EKG changes. CTG length was not correlated with the frequency of EKG abnormalities, but was inversely correlated with the age at onset of EKG abnormalities (p < 0.0001). CTG length influences the timing of cardiac complications in myotonic dystrophy.
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页码:1207 / 1209
页数:3
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