Sinus node paucity in hyperekplexia
Indian Pediatrics, cilt.47, sa.12, ss.1066-1068, 2010 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 47 Sayı: 12
- Basım Tarihi: 2010
- Doi Numarası: 10.1007/s13312-010-0165-4
- Dergi Adı: Indian Pediatrics
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.1066-1068
- Anahtar Kelimeler: Hyperekplexia, Neonate, Seizures, Sinus node
- Sağlık Bilimleri Üniversitesi Adresli: Evet
Özet
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. Continous 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 occured. 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. © 2010 Indian Academy of Pediatrics.