Epidemiology and clinical spectrum of schizencephaly in South-Eastern Hungary

Nóra Szabó, Gergev Gyurgyinka, Jeno Kóbor, Edit Bereg, Sándor Túri, László Sztriha

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4 Citations (Scopus)

Abstract

The epidemiology and clinical spectrum of schizencephaly in south-eastern Hungary have been surveyed in a retrospective population-based study. A total of 10 patients (6 boys and 4 girls) were found with schizencephaly among 185 486 live births in a period of 14 years (July 1, 1992 to June 30, 2006), which means a birth prevalence of 0.54 per 10 000 (95% confidence interval [CI]: 0.20-0.87). The schizencephaly was unilateral in 7 cases (with closed lips in 5 and open lips in 2 patients) and bilateral in 3 children (with closed lips in 2 and open lips in 1). The septum pellucidum was absent in 5 cases; however, optic nerve hypoplasia was not found in these patients. Delayed development and intellectual disability were observed in all patients, except 2 with unilateral closed lip schizencephaly. Epilepsy was diagnosed in 3 patients (2 with unilateral and 1 with bilateral schizencephaly).

Original languageEnglish
Pages (from-to)1335-1339
Number of pages5
JournalJournal of Child Neurology
Volume25
Issue number11
DOIs
Publication statusPublished - Nov 1 2010

Keywords

  • cortical malformation
  • developmental delay
  • epidemiology
  • epilepsy
  • intellectual disability
  • polymicrogyria
  • schizencephaly

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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