Literature DB >> 24408398

Clinical profile of pediatric patients with long QT syndrome masquerading as seizures.

Bhavesh Thakkar1, Anand Shukla, Tarandeep Singh, Saurin Shah, Shomu Bohora, Jayal Shah, Tarun Madan.   

Abstract

OBJECTIVE: To study the clinical profile of patients with long QT syndrome presenting as seizures.
METHODS: Retrospective analysis of six pediatric patients admitted at authors' institute between October 2008 and January 2013 with seizures and a presumptive diagnosis of long QT syndrome (LQTS) was done. The diagnosis was made on the basis of updated Schwartz diagnostic criteria. Clinical data, investigation profile and follow up of patients was recorded in a standard format and analysed.
RESULTS: All the 6 patients in the study were boys with a mean age of 10.3 ± 2.8 y at the time of diagnosis. The lag period between symptom onset and diagnosis was 5.6 ± 3.14 y. All patients had history of seizures with a history of precipitating event in 4 patients. Average baseline QTc interval was 556 ± 41.31 ms. Mean Schwartz score was 6.66 ± 1.16. Polymorphic VT was documented in 4 patients. After initiating standard treatment with betablockers, nicorandil, spironolactone or pacemaker, all the six patients were asymptomatic at a mean follow up period of 17.5 mo, with no recurrence of seizures.
CONCLUSIONS: LQTS can cause seizures due to prolonged ventricular arrhythmias in high risk subgroup. Children, who present with LQTS and seizures, generally have a precipitating event causing seizures, and they respond well to drug therapy.

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Year:  2014        PMID: 24408398     DOI: 10.1007/s12098-013-1305-3

Source DB:  PubMed          Journal:  Indian J Pediatr        ISSN: 0019-5456            Impact factor:   1.967


  24 in total

1.  Electrocardiographic prediction of abnormal genotype in congenital long QT syndrome: experience in 101 related family members.

Authors:  E S Kaufman; S G Priori; C Napolitano; P J Schwartz; S Iyengar; R C Elston; A H Schnell; E Z Gorodeski; G Rammohan; N O Bahhur; D Connuck; L Verrilli; D S Rosenbaum; A M Brown
Journal:  J Cardiovasc Electrophysiol       Date:  2001-04

Review 2.  The congenital long QT syndromes from genotype to phenotype: clinical implications.

Authors:  Peter J Schwartz
Journal:  J Intern Med       Date:  2006-01       Impact factor: 8.989

3.  Macroscopic T wave alternans in long QT syndrome.

Authors:  M P Jayakrishnan; P Krishnakumar
Journal:  Indian J Pediatr       Date:  2006-06       Impact factor: 1.967

4.  Jervell-Lange Nielsen syndrome in a family with the long QT Syndrome (LQTS).

Authors:  R K Mondal; B Karmakar; P K Chandra; U N Sarkar
Journal:  Indian J Pediatr       Date:  2006-07       Impact factor: 1.967

5.  Idiopathic long QT syndrome: progress and questions.

Authors:  P J Schwartz
Journal:  Am Heart J       Date:  1985-02       Impact factor: 4.749

6.  Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing.

Authors:  David J Tester; Melissa L Will; Carla M Haglund; Michael J Ackerman
Journal:  Heart Rhythm       Date:  2005-05       Impact factor: 6.343

7.  Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2.

Authors:  I Splawski; J Shen; K W Timothy; M H Lehmann; S Priori; J L Robinson; A J Moss; P J Schwartz; J A Towbin; G M Vincent; M T Keating
Journal:  Circulation       Date:  2000-09-05       Impact factor: 29.690

Review 8.  Long QT syndrome manifesting as pulseless epilepsy.

Authors:  Fuad Abed Abass; Madhukar Shahi; Nirmal Kumar; Moneesha Bhargava; Sangita Gupta; Jacob M Puliyel
Journal:  Indian J Pediatr       Date:  2003-01       Impact factor: 1.967

9.  The congenital long QT syndromes in childhood.

Authors:  R G Weintraub; R M Gow; J L Wilkinson
Journal:  J Am Coll Cardiol       Date:  1990-09       Impact factor: 24.094

10.  Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry.

Authors:  E H Locati; W Zareba; A J Moss; P J Schwartz; G M Vincent; M H Lehmann; J A Towbin; S G Priori; C Napolitano; J L Robinson; M Andrews; K Timothy; W J Hall
Journal:  Circulation       Date:  1998-06-09       Impact factor: 29.690

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  1 in total

1.  Nicorandil, a Nitric Oxide Donor and ATP-Sensitive Potassium Channel Opener, Protects Against Dystrophin-Deficient Cardiomyopathy.

Authors:  Muhammad Z Afzal; Melanie Reiter; Courtney Gastonguay; Jered V McGivern; Xuan Guan; Zhi-Dong Ge; David L Mack; Martin K Childers; Allison D Ebert; Jennifer L Strande
Journal:  J Cardiovasc Pharmacol Ther       Date:  2016-03-02       Impact factor: 2.457

  1 in total

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