The prolonged QT syndrome presenting as epilepsy: A report of two cases and literature review

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Pacia SV, Devinsky O, Luciano DJ, and Vazquez B (1994) The prolonged QT syndrome presenting as epilepsy: A report of two cases and literature review. Neurology 44:8 1408–10.

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Abstract: 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.

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  • Case report describing two patients with long QT syndrome and reviews 8 previously presented cases, finding that ‘lifelessness’ prior to seizure activity was a common finding. Beta-blockers appeared to be effective in preventing recurrences. Gatto et al. comment in a letter in response to this article that EEG should include EKG on one channel to permit detection of cardiogenic seizures.

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