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The prolonged QT syndrome presenting as epilepsy

A report of two cases and literature review

Department of Neurology and Hospital for Joint Diseases Epilepsy Center, New York University, New York, NY.

The prolonged QT syndrome is associated with ventricular tachyarrhythmias and sudden death. We report two patients and review eight previously reported cases of this syndrome, presenting as epilepsy. The average age at the time of the first convulsion was 4.7 years. Episodes were often infrequent, and the time to correct diagnosis ranged from 1 to 28 years. Only one-half the patients had histories suggestive of a familial syndrome. Presyncopal complaints and "lifelessness" prior to seizure activity were common findings in retrospect. Beta-blockade was effective in preventing recurrences in all patients who received treatment.

Address correspondence and reprint requests to Dr. Steven Pacia, Department of Neurology, Hospital for Joint Diseases, 301 East 17th Street, New York, NY 10003.

Received November 2, 1993. Accepted in final form February 4, 1994.