how to cite this article: momen aa. momen m. double cortex syndrome (subcortical band heterotopia); a case report. iran j child neurol. 2015;9(2):64-68. abstract objective approximately 5–10% of preschool age children are considered developmentally disabled. brain magnetic resonance imaging (mri) plays a key role in the diagnostic evaluation in these children. many congenital or acquired brain ...

The malformations of the cerebral cortex represent a major cause of developmental disabilities, severe epilepsy and reproductive disadvantage. The advent of high resolution MRI techniques has facilitated the in vivo identification of a large group of cortical malformation phenotypes. Several malformation syndromes caused by abnormal cortical development have been recognized and specific causati...

A girl with Klippel-Trenaunay-Weber syndrome with partial motor seizures is reported. She had hemimegalencephaly and band heterotopia on MRI of the brain.

In 1989, Barkovich et al. [1] described a new neuronal migration anomaly referred to thereafter as band heterotopia. Similar findings were also reported by Livingston and Aicardi [2] in describing two children with mild epilepsy and learning behavior problems. In band heterotopia, a continuous band of heterotopic neurons is interposed between the lateral ventricles and the cerebral cortex. The ...

X-SCLH/LIS syndrome is a neuronal migration disorder with disruption of the six-layered neocortex. It consists of subcortical laminar heterotopia (SCLH, band heterotopia, or double cortex) in females and lissencephaly (LIS) in males, leading to epilepsy and cognitive impairment. We report the characterization of a novel CNS gene encoding a 40 kDa predicted protein that we named Doublecortin and...

OBJECTIVE Subcortical band heterotopia (SBH) is a cortical malformation formed when neocortical neurons prematurely stop their migration in the white matter, forming a heterotopic band below the normotopic cortex, and is generally associated with intractable epilepsy. Although it is clear that the band heterotopia and the overlying cortex both contribute to creating an abnormal circuit prone to...

Objective Approximately 5-10% of preschool age children are considered developmentally disabled. Brain Magnetic Resonance Imaging (MRI) plays a key role in the diagnostic evaluation in these children. Many congenital or acquired brain anomalies are revealed with MRIs. Although the majority of these abnormalities are sporadic but patients with subcortical band heterotopia or double cortex syndro...

During brain development, radial glial cells possess an apico-basal polarity and are coupled by adherens junctions (AJs) to an F-actin belt. To elucidate the role of the actin, we conditionally deleted the key component α-E-catenin in the developing cerebral cortex. Deletion at early stages resulted in severe disruption of tissue polarity due to uncoupling of AJs with the intracellular actin fi...

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...