Disease relevance of Septum Pellucidum

• The authors report a series of 10 low-grade neoplasms arising in the midline anteriorly in the region of the septum pellucidum with many of the histologic features of dysembryoplastic neuroepithelial tumor (DNT) [1].
• Patients with muscle-eye-brain disease had cerebellar polymicrogyria (with or without cysts), absence of the septum pellucidum, diffuse cerebral cortical dysplasia, pontine and cerebellar vermian hypoplasia, patchy hypomyelination, and variable callosal hypogenesis and hydrocephalus [2].
• Autopsy revealed a subependymal giant cell astrocytoma of the septum pellucidum, but there were no other lesions of tuberous sclerosis [3].
• The clinical triad of Septo-optic dysplasia (SOD) comprises the absence of the septum pellucidum, congenital optic nerve dysplasia, and multiple endocrine defects [4].
• Sudden death in epilepsy due to an isolated subependymal giant cell astrocytoma of the septum pellucidum [3].

High impact information on Septum Pellucidum

• On qualitative analysis, we found a high incidence of midline developmental abnormalities (cavum septum pellucidum, or CSP) [5].
• All patients had clinical (stupor or coma, loss of pontomesencephalic brainstem reflexes, extensor posturing) or radiologic (midline shift of septum pellucidum > or =1 cm downward or obliteration of the ambient and suprasellar cisterns caused by displacement of the temporal uncus) signs of herniation [6].
• Growth hormone deficiency associated with the ectrodactyly-ectodermal dysplasia-clefting syndrome and isolated absent septum pellucidum [7].
• A giant septum pellucidum cyst was demonstrated on Conray ventriculography and later on computerized tomography (CT) scanning [8].
• Thirty-two female (mean age=52.9 years [SD=9.2]) patients with a diagnosis of residual schizophrenia and 19 female (mean age=51.1 years [SD=12.7]) control subjects were studied through cerebral Magnetic Resonance Imaging. Along the entire surface of the septum pellucidum, 1-mm coronal slices were performed in all subjects [9].

Anatomical context of Septum Pellucidum

• Readers rated corpus callosum involvement, midline spread, subependymal spread, new discrete multiple enhancing foci, a "spreading wavefront" appearance, and septum pellucidum involvement [10].
• Mental retardatin and epilepsy, in addition to absence of the septum pellucidum, point to a widespread lesion of the central nervous system [11].
• The authors present two cases of schizophrenia in young males who have malformations of the septum pellucidum, a thin sheet of cells and fibers located in the midline of the brain that separates the lateral ventricles [12].
• This study looked at the BPD utilizing coronal (anatomically at right angles to the traditional BPD through the thalami, third ventricle, and cavum septum pellucidum) rather than transverse section through the fetal head [13].

Associations of Septum Pellucidum with chemical compounds

• CONCLUSIONS: To our knowledge, our patient represents the second reported case of anomalous septum pellucidum after intrauterine valproate exposure [14].
• This case suggests the existence of some stimulatory fiber for the secretion of luteinizing hormone in the septum pellucidum [15].

Gene context of Septum Pellucidum

• An anomaly of the septum pellucidum, a so-called cavum veli interpositi was found in the psychotic twin while his brother showed no such anomaly [16].
• Only a few cases of symptomatic non-communicating cava, called septum pellucidum and cavum Vergae cysts, have been described in the literature [17].
• Also other rare tumors reminding DNT have been reported: DNT-like neoplasm of septum pellucidum and rosetted glioneuronal tumor [18].
• Panic disorder is thought to involve dysfunction in the septohippocampal system, and the presence of a cavum septum pellucidum might indicate the aberrant development of this system [19].
• Only one sister, who presented with microcephaly and developmental delay, showed signs of dysplasia of the midline structures (ie, septum pellucidum and corpus callosum) and a large intracranial cyst [20].

Analytical, diagnostic and therapeutic context of Septum Pellucidum

• In a retrospective study of 86 cases with NF1 we found three patients with septum pellucidum hamartomas (frequency = 3.4 %) [21].

References