Familial Q-T Prolongation Syndrome

Singer, Philip A.

doi:10.1001/archneur.1974.00490370090017

Arch Neurol

1974

DOI: 10.1001/archneur.1974.00490370090017

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Familial Q-T Prolongation Syndrome

Philip A. Singer¹

Abstract: A young woman with familial prolonga-

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Cited by 25 publications

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Hereditary long Q‐T syndrome presenting as epilepsy: Electroencephalography laboratory diagnosis

Gospe¹,

Choy²

1989

Annals of Neurology

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Patients with hereditary Q-T interval prolongation can present with seizures, syncope, and sudden death. In 2 siblings with autosomal dominant familial long Q-T syndrome, electroencephalographic examinations performed 6 and 2 years before diagnosis included electrocardiographic tracings documenting the cardiac abnormality. A timely diagnosis of this condition may have prevented the death of 1 of these patients. Measurement of the corrected Q-T interval on electrocardiographic tracings obtained in the electroencephalography laboratory should be considered in selected patients.

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Hereditary long Q‐T syndrome presenting as epilepsy: Electroencephalography laboratory diagnosis

Gospe¹,

Choy²

1989

Annals of Neurology

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Current Concepts in Long QT Syndrome

Fuentes-Garcia

Towbin

2000

Pediatr Cardiol

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Sudden cardiac death occurs in the United States with an incidence of more than 300,000 persons per year. The underlying cause of death is commonly considered to be due to primary or secondary arrhythmias. In young persons in whom no structural heart disease can be identified, the long QT syndromes (LQTS) are commonly considered as likely causes. Multiple genes causing LQTS have been identified thus far, all of which encode cardiac ion channels. These include two potassium channel alpha subunits (KVLQT1 and HERG), two potassium channel beta subunits (minK and MiRP1), and one sodium channel gene (SCN5A). The purpose of this review is to describe the current understanding of the molecular genetics of LQTS and the resultant phenotypes, particularly in young patients.

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Epilepsie- und Long-QT-Syndrom?Fallvorstellung

Rimpau

Jäger‐Roman

Köpcke³

2004

Zeitschrift f�r Epileptologie

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Epilepsy and long QT-syndromeCase reportn Abstract In a Kurdish female patient, an idiopathic long QTsyndrome (LQTS) was diagnosed after twenty years of symptoms. Within two generations of her extended family, there were fifteen afflicted members. With this case, we present an uncommon occurrence of a seizure disorder with syncope and epileptic seizures from LQTS. A newly discovered mutation of the KCNQ1-protein seems to be responsible for the combined appearance of symptoms from cardiac arrhythmias and epileptic seizures. We discuss the literature with regard to the complex differential diagnosis of LQTS with syncope implied loss of consciousness and postural tone, syncopal myoclonus and grand mal seizures.

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Familial Q-T Prolongation Syndrome

Abstract: A young woman with familial prolonga-

Cited by 25 publications

References 30 publications

Hereditary long Q‐T syndrome presenting as epilepsy: Electroencephalography laboratory diagnosis

Hereditary long Q‐T syndrome presenting as epilepsy: Electroencephalography laboratory diagnosis

Current Concepts in Long QT Syndrome

Epilepsie- und Long-QT-Syndrom?Fallvorstellung

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