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Sinus node paucity in hyperekplexia.
MedLine Citation:
PMID:  21220806     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
We report a newborn with hyperekplexia and uncontrolled tonic spasms which did not respond to intravenous phenobarbitone and phenytoin, and midazolam infusion. Serum biochemistry, electrocardiography, electroencephalography, lumbar puncture and neuroimaging were normal. Continuous cardiac monitoring revealed that tonic spasm episodes were accompanied by sinus node paucity and severe bradycardia. Duration and number of tonic spasm episodes decreased with clonazepam therapy, and she was discharged. At 4 months of age sudden infant death occurred. Sudden infant death could be related to the paucity of sinus node. Cardiac pacemaker implantation should be considered even if the medical treatment is successful.
Authors:
S Ozkiraz; Z Gokmen; U A Orün; F Alehan
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Indian pediatrics     Volume:  47     ISSN:  0974-7559     ISO Abbreviation:  Indian Pediatr     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2011-01-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985062R     Medline TA:  Indian Pediatr     Country:  India    
Other Details:
Languages:  eng     Pagination:  1066-8     Citation Subset:  IM    
Affiliation:
Department of Neonatology, Medical School of Baskent University, Ankara, Turkey. sozKiraz@yahoo.com
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