Over the past decade enhanced activation of the mammalian target of rapamycin (mTOR)-signaling cascade has been identified in focal malformations of cortical development (MCD) subtypes, which have been collectively referred to as "mTORopathies." Mutations in mTOR regulatory genes (e.g., TSC1, TSC2, AKT3, DEPDC5) have been associated with several focal MCD highly associated with epilepsy such as...

This review surveys immunocytochemical and histochemical markers of neuronal lineage for application to tissue sections of fetal and neonatal brain. They determine maturation of individual nerve cells as the tissue progresses to mature architecture. From a developmental perspective, neuronal markers are all about timing. These diverse cellular labels may be classified in two ways: 1) time of on...

Nearly 68% of patients with cutis marmorata have a congenital abnormality, the most common being body asymmetry. Cutis marmorata has also been shown to be associated with the chiari malformations, macrocephaly, hemimegalencephaly, bilateral cortical dysplasia, calvarial haemangioma and cavum septi pellucidum cysts.2 In fact, this subset of patients may have developmental delay and are now class...

PURPOSE The aim of the study was to report the seizure outcome, motor skills and adaptive motor functions in a series of children and adolescents who underwent hemispheric surgery, analysing the risk-benefits of surgery. METHODS The clinical course, seizure and motor function outcomes of 15 patients who underwent hemispheric surgery were reviewed. RESULTS The mean age at surgery was 9.5, wi...

OBJECTIVE To record the pattern of different neuronal migrational disorders (NMD) and their associated neurological conditions. METHODS The data were collected at the Child Neurology Services of Sultan Qaboos University Hospital, Oman, from January 1993 to September 2006 from all children with psychomotor delay and epilepsy, who underwent brain imaging (mostly MRI). The MR imaging was used fo...

Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neur...

OBJECTIVES To study the outcome of disconnective epilepsy surgery for intractable hemispheric and sub-hemispheric pediatric epilepsy. METHODS A retrospective analysis of the epilepsy surgery database was done in all children (age <18 years) who underwent a peri-insular hemispherotomy (PIH) or a peri-insular posterior quadrantectomy (PIPQ) from April 2000 to March 2011. All patients underwent ...

− Classification is a creative activity that helps us understand relationships. The traditional classifications of central nervous system malformations was based exclusively upon descriptive morphology, but these criteria must now be integrated with molecular genetic data to enable an etiological classification that also remains useful to the clinician, radiologist and pathologist, who rely upo...

Background Attention deficit and hyperactivity disorder (ADHD) is a disorder of the brain characterized by periods of inattention, hyperactivity and impulsive behavior. We aimed to evaluate the role of MRI of the brain in children with ADHD. Materials and Methods This prospective study included 100 children with clinical diagnosis of attention deficit and hyperactivity disorder according to the...