INTRODUCTION Under-five mortality estimates are increasingly used in low and middle income countries to target interventions and measure performance against global development goals. Two new methods to rapidly estimate under-5 mortality based on Summary Birth Histories (SBH) were described in a previous paper and tested with data available. This analysis tests the methods using data appropriate...

Derzhavin (1896) described the first recorded case, with a vascularized band extending from the whole width of the optic disc to the periphery of the retina, which at first formed a retinal fold and then broke free from the retina as a band extending forwards into the vitreous. It has been suggested that this represented remnants of the hyaloid artery. Grondahl (1963) has reported a child with ...

Rho-GTPases have been found to be crucial for cytoskeleton remodelling and cell polarity, as well as key players in directed cell migration in various tissues and organs, therefore becoming good candidates for involvement in neuronal migration disorders. We recently found that genetic deletion of the small GTPase RhoA in the developing mouse cerebral cortex results in three distinct cortical ma...

Radial glial cells (RGCs) in the ventricular neuroepithelium of the dorsal telencephalon are the progenitor cells for neocortical projection neurons and astrocytes. Here we show that the adherens junction proteins afadin and CDH2 are critical for the control of cell proliferation in the dorsal telencephalon and for the formation of its normal laminar structure. Inactivation of afadin or CDH2 in...

The neuronal migration disorders, X-linked lissencephaly syndrome (XLIS) and subcortical band heterotopia (SBH), also called "double cortex", have been linked to missense, nonsense, aberrant splicing, deletion, and insertion mutations in doublecortin (DCX) in families and sporadic cases. Most DCX mutations identified to date are located in two evolutionarily conserved domains. We performed muta...

BACKGROUND Periventricular nodular heterotopia (PNH) is a rare congenital anomaly of the brain presenting as nodular heterotopia along the paraventricular region. Ten cases of PNH complicated by aortic aneurysm have been reported in the literature, and 9 of them also had symptoms of Ehlers-Danlos syndrome (EDS). This study investigated the association of PNH and cardiovascular anomalies in Asia...

PURPOSE To investigate the clinical syndrome and associated brain anomalies in a group of patients with subcortical heterotopia. METHODS The seizure history, developmental history, family history, electroencephalographic results, imaging studies, and pathologic results (where available) of 13 patients with subcortical heterotopia were reviewed retrospectively. The patients ranged in age from ...

we report an unusual case of hemimegalencephaly (hmg) associated with ipsilateral congenital-infiltrating lipomatosis of the face in a five-month-old boy. hemimegalencephaly is a rare but unique malformation characterized by enlargement of all or parts of a cerebral hemisphere. the affected hemisphere may have focal or diffuse neuronal migration defects, with areas of polymicrogyria, pachygyria...

Subcortical band heterotopia (SBH), also known as ‘double cortex’ syndrome, is characterized by a band of heterotopic neurons interspersed in the white matter between the cortex and the lateral ventricles and is usually a severe disease with patients showing variable degrees of developmental delay, intellectual disability, motor impairment and epilepsy [1]. This is a true neuronal migration dis...