Epidemiology and clinical spectrum of schizencephaly in south-eastern Hungary.
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).
Szabó
Nóra
N
Department of Paediatrics, University of Szeged, Szeged, Hungary.
Gyurgyinka
Gergev
G
Kóbor
Jeno
J
Bereg
Edit
E
Túri
Sándor
S
Sztriha
László
L
D002658:Developmental Disabilities / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D008607:Intellectual Disability / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D054220:Malformations of Cortical Development / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology; D015995:Prevalence; D012189:Retrospective Studies; D012688:Septum Pellucidum / Q000002:abnormalities