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Septo-optic dysplasia (SOD), known also as de Morsier syndrome, is a rare congenital malformation syndrome that features a combination of the underdevelopment of the optic nerve, pituitary gland dysfunction, and absence of the septum pellucidum (a midline part of the brain).
Motor delay is most common (75%) and communication delay is least common (44%). Predictors of significantly delayed development include hypoplasia or agenesis of the corpus callosum and hypothyroidism. The absence of the septum pellucidum does not predict developmental delay. Delays may occur in unilateral (39%) as well as bilateral (78%) cases ...
The both of them exhibited bilateral porencephaly, an underdeveloped cerebellum, an absent vermis, an absent septum pellucidum, and generalized internal malformations, most of which were unique to one another; [6]
The septum pellucidum (Latin for "translucent wall") is a thin, triangular, vertical double membrane separating the anterior horns of the left and right lateral ventricles of the brain. It runs as a sheet from the corpus callosum down to the fornix. The septum is not present in the syndrome septo-optic dysplasia.
Septum Verum (true septum) is a region in the lower medial part of the telencephalon that separates the two cerebral hemispheres.. The human septum consists of two parts: the septum pellucidum (translucent septum), a thin membrane consisting of white matter and glial cells that separate the lateral ventricles, and the lower, precommisural septum verum, which consists of nuclei and grey matter.
The septal area refers to the nearby septum pellucidum. It is located underneath the corpus callosum and in front of the lamina terminalis . The lamina terminalis is a layer of gray matter that connects the optic chiasma and the anterior commissure . [ 7 ]
Separating the anterior horns of the lateral ventricles is the septum pellucidum: a thin, triangular, vertical membrane which runs as a sheet from the corpus callosum down to the fornix. During the third month of fetal development, a space forms between two septal laminae, known as the cave of septum pellucidum (CSP), which is a marker for ...
Rhombencephalosynapsis is an anomaly characterized by the absence or severe dysgenesis of the cerebellar vermis with fusion of the cerebellar hemispheres, peduncles, and dentate nuclei. Diagnostic features include fusion of the midbrain colliculi , hydrocephalus , absence of the corpus callosum other midline structural brain malformations.