title: Corpus callosum anomalies: birth prevalence and clinical spectrum in Hungary.
abstract: Data regarding the epidemiology of callosal anomalies are contradictory. We performed a population-based retrospective survey to study the birth prevalence and clinical features of agenesis/hypoplasia of the corpus callosum and accompanying central nervous system and somatic abnormalities in southeastern Hungary between July 1, 1992 and June 30, 2006. Among 185,486 live births, 38 patients (26 boys and 12 girls) manifested agenesis/hypoplasia of the corpus callosum, corresponding to a prevalence of 2.05 per 10,000 live births (95% confidence interval, 1.4-2.7). Callosal anomalies were isolated in 18 patients, and were associated with other central nervous system malformations in five children. Both central nervous system and noncentral nervous system abnormalities were evident in seven patients, whereas callosal dysgenesis was accompanied only by somatic anomalies in eight children. Five of 18 patients with isolated agenesis/hypoplasia of the corpus callosum remained asymptomatic. Developmental delay, intellectual disability, or epilepsy occurred in all patients, except one, when callosal anomalies were combined with other brain or somatic abnormalities. Five patients with multiplex malformations died. Callosal anomalies form a clinically significant and relatively frequent group of central nervous system malformations.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, University of Szeged, Szeged, Hungary.
authors.forename: Nóra
authors.initials: N
authors.lastname: Szabó
authors.forename: Gyurgyinka
authors.initials: G
authors.lastname: Gergev
authors.forename: Jeno
authors.initials: J
authors.lastname: Kóbor
authors.forename: Edit
authors.initials: E
authors.lastname: Bereg
authors.forename: Sándor
authors.initials: S
authors.lastname: Túri
authors.forename: László
authors.initials: L
authors.lastname: Sztriha
mesh_terms: D000293:Adolescent; D061085:Agenesis of Corpus Callosum / Q000175:diagnosis / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D003337:Corpus Callosum / Q000473:pathology; D016208:Databases, Factual; D005260:Female; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D007223:Infant; D050498:Live Birth / Q000453:epidemiology*; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies