Clinical and morphological analysis of cortical dysplasias accompanied by epileptic syndromes and symptomatic epilepsy in children

Clinical and morphological correlations in the group of cortical dysplasias accompanied by the development of an epileptic syndrome were performed based on 50 autopsies. The diagnosis of microcephaly was confirmed in 40 patients who died; polymicrogyria was detected in 6 cases; holoprosencephaly was detected in 4 cases. Analysis of the consequences of paroxysmal neurological disorders revealed that the West syndrome in the first year of life dominated in 32% of died patients with cortical dysplasias, myoclonic epilepsy of infancy in 4% of the cases, and the Ohtahara syndrome in 4% of the cases. The severity of epilepsy progression was determined by the onset age and the structure of epileptic seizures. Motor and cognitive disorders of varying severity were noted in all patients with cortical dysplasias. Associated developmental anomalies of the internal organs were found in 100% of the cases. Microcephaly was combined with other congenital brain anomalies in 62.5% of the cases, while polymicrogyria and holoprosencephaly in 100% of the cases. A histological examination of the brain of dead patients with microcephaly, polymicrogyria, and holoprosencephaly revealed a number of common features, including a reduction in the number and density of neurons, abnormalities of the cortical cytoarchitecture, and the presence of large dysmorphic neurons. These findings confirm the fact that these forms of pathology belong to the group of cortical dysplasias.