An unusual cause of apparent epilepsy: ECG and EEG findings in a case of Jervell Lange-Neilson syndrome.
نویسندگان
چکیده
A case is presented of apparent epilepsy which proved to be due to recurrent ventricular tachyarrhythmias (torsade de pointe). The relationship between the cardiac arrhythmia and changes in the electroencephalograph is recorded and analysed. This is probably an example of the 'Jervell Lange-Neilson' syndrome of cardiac arrhythmias which may produce ictal episodes, prolongation of the QTc interval of the ECG, and sensori-neural deafness. The features of the syndrome, its pathology and treatment, and its relevance to the mangement of epilepsy are discussed.
منابع مشابه
Novel frameshift mutation in the KCNQ1 gene responsible for Jervell and Lange-Nielsen syndrome
Objective(s): Jervell and Lange–Nielsen syndrome is an autosomal recessive disorder caused by mutations in KCNQ1 or KCNE1 genes. The disease is characterized by sensorineural hearing loss and long QT syndrome. Methods: Here we present a 3.5-year-old female patient, an offspring of consanguineous marriage, who had a history of recurrent syncope and congenital sensorineural deafness. The patient ...
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Long QT syndrome, which is defined by corrected QT interval longer than 0.45 seconds in men and o. 47 sec in women , could be divided into idiopathic (congenital ) and acquired forms. The idiopathic form is a familial disorder that can be associated with sensorineural deafness (Jervell and Lange- Neelson syndrome), which is transmitted with an autosomal recessive pattern. Although this syndrome...
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A26-year-old woman with congenital deafness and a lifelong “seizure disorder” presented to the emergency department with 15 episodes of syncope. She was 1 week postpartum. The ECG showed normal sinus rhythm, markedly prolonged QT interval (900 ms), and remarkably large and broad T waves (Figure). In the emergency room, the patient had another syncopal episode associated with tonic-clonic activi...
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عنوان ژورنال:
- Journal of neurology, neurosurgery, and psychiatry
دوره 40 11 شماره
صفحات -
تاریخ انتشار 1977