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Neurology 2000;55:1207-1209
© 2000 American Academy of Neurology
Brief Communications

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

G. Antonini, MD, F. Giubilei, MD, PhD, A. Mammarella, MD, P. Amicucci, PhD, M. Fiorelli, MD, PhD, F. Gragnani, MD, S. Morino, MD, PhD, V. Ceschin, MD, P. V. Fragola, MD and M. Gennarelli

From the Departments of Neurological Sciences (Drs. Antonini, Giubilei, Fiorelli, Gragnani, Morino, and Ceschin) and Medical Therapy (Drs. Mammarella and Fragola), University of Rome "La Sapienza"; and the Department of Biopathology and Diagnostic Imaging (Dr. Amicucci and M. Gennarelli), University of Rome "Tor Vergata," Italy.

Address correspondence and reprint requests to Dr. Giovanni Antonini, Department of Neurological Sciences, Viale dell’Università 30, 00185 Rome, Italy; e-mail: antoninimusc{at}uniroma1.it

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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