CONTEXT Although the causes of some malformations of cortical development (MCD) have been established, others remain unclear. There are several lines of evidence supporting the theory of a complex mechanism that involves genetic and environmental factors. OBJECTIVE To investigate the interrelationship of genetics and prenatal injury in the genesis of MCD. PATIENTS AND DESIGN A series of 76 ...

A case of congenital bilateral perisylvian syndrome (CBPS) associated with bilateral perisylvian schizencephaly in a 24-year-old woman is reported. She presented the classical clinical triad of CBPS, which included congenital facio-masticatory diplegia, epilepsy and only mild mental retardation, despite the presence of bilateral, open-lip clefts in the perisylvian region. We hypothesize that th...

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The coexistence of band heterotopia and polymicrogyria is extremely rare though it has been reported in the presence of corpus callosum anomalies and megalencephaly. We present prenatal and postnatal MRI findings of a rare case of diffuse cortical malformation characterized by polymicrogyria and band heterotopia. Agenesis of the corpus callosum and megalencephaly were also noted. In addition, b...

In a review of more than 2000 MR images of the brain we identified 35 patients with absence of the septum pellucidum. These patients were divided into seven basic groups as follows: septooptic dysplasia; schizencephaly; holoprosencephaly; agenesis of the corpus callosum; chronic, severe hydrocephalus; basilar encephaloceles; and porencephaly/hydranencephaly. Absence of the septum pellucidum was...

BACKGROUND Gray matter heterotopia (GMH) is a malformation of the central nervous system characterized by interruption of normal neuroblasts migration between the 7(th) and 16(th) week of fetal development. The aim of the study was the analysis of clinical symptoms, prevalence rate and the most common concurrent central nervous system (CNS) developmental disorders as well as assessment of chara...