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Early Postnatal Seizures in a Neonate with Wolf-Hirschhorn Syndrome

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Journal AJP Rep
Date 2016 Dec 21
PMID 27994945
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Abstract

 Wolf-Hirschhorn syndrome (WHS), which is characterized by a typical facial appearance, growth retardation, mental retardation, seizures, and congenital cardiac defects, has an estimated incidence of 1 per 50,000 births.  We report a case of a low birth weight neonate with WHS and seizures, as well as persistent pulmonary hypertension in the early neonatal period. Apgar scores were 6 (1 minute) and 8 (5 minutes) with evident retraction. After admission to the neonatal intensive care unit, the patient had tonic-clonic seizures with epilepticus 30 minute after birth. Although the seizures were uncontrollable, continuous thiopental administration was effective for seizure mitigation.  Neonatal seizures with WHS occur rarely. This is the first case report on seizures just after birth in a neonate with WHS.

References
1.
Garg D, Goldberg R, Weidler D . Pharmacokinetics of thiopental in the asphyxiated neonate. Dev Pharmacol Ther. 1988; 11(4):213-8. DOI: 10.1159/000457691. View

2.
Scher M, Klesh K, Murphy T, Guthrie R . Seizures and infarction in neonates with persistent pulmonary hypertension. Pediatr Neurol. 1986; 2(6):332-9. DOI: 10.1016/0887-8994(86)90073-1. View

3.
Silverstein F, Jensen F . Neonatal seizures. Ann Neurol. 2007; 62(2):112-20. DOI: 10.1002/ana.21167. View

4.
Shannon N, Maltby E, Rigby A, Quarrell O . An epidemiological study of Wolf-Hirschhorn syndrome: life expectancy and cause of mortality. J Med Genet. 2001; 38(10):674-9. PMC: 1734744. DOI: 10.1136/jmg.38.10.674. View

5.
Battaglia A, Carey J, Wright T . Wolf-Hirschhorn (4p-) syndrome. Adv Pediatr. 2001; 48:75-113. View