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 Volume 63, Number 10, November 23, 2004
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NEUROLOGY 2004;63:1939-1941
© 2004 American Academy of Neurology
Brief Communications

Severe cardiac arrhythmias in young patients with myotonic dystrophy type 1

G. Bassez, MD, A. Lazarus, MD, I. Desguerre, MD, J. Varin, MD, P. Laforêt, MD, H. M. Bécane, MD, C. Meune, MD, M. C. Arne-Bes, MD, Z. Ounnoughene, MD, H. Radvanyi, MD, PhD, B. Eymard, MD, PhD and D. Duboc, MD, PhD

From the Myology Institute (Drs. Bassez, Laforêt, Bécane, and Eymard), Groupe Hospitalier Pitié-Salpêtrière, Paris; Department of Histology (Dr. Bassez), Hôpital Henri Mondor, Créteil; Department of Cardiology (Drs. Lazarus, Varin, Meune, Ounnoughene, and Duboc), Hôpital Cochin, Paris; Department of Pediatrics (Dr. Desguerre), Child Neurology Unit, Hôpital Necker, Paris; Department of Neurology (Dr. Arne-Bes), Hôpital Rangueil, Toulouse; and Department of Molecular Genetics (Dr. Radvanyi), Hôpital Ambroise Paré, Boulogne, France.

Address correspondence and reprint requests to Dr. Denis Duboc, Department of Cardiology, Hôpital Cochin, 27 rue du Faubourg Saint Jacques, 75679 Paris Cedex 14, France; e-mail: denis.duboc{at}cch.ap-hop-paris.fr

Cardiac tachyarrhythmias have rarely been studied in young patients with myotonic dystrophy type 1 (DM1). The authors observed major cardiac rhythm disturbances in 11 patients aged 10 to 18 years. Tachyarrhythmic events were more frequent than impulse conduction disorders. Wide variations in CTG expansion were observed among the population. Since physical exercise was a prominent arrhythmogenic factor, systematic exercise tests with EKG monitoring may be indicated in young patients with DM1.

Received February 7, 2004. Accepted in final form July 9, 2004.

Presented in part at the 54th annual meeting of the American Academy of Neurology; April 13–20, 2002.




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