Isolated absence of the septum pellucidum

Absence of the septum pellucidum in the human is a rare congenital anomaly. Previous reports suggest it is almost always associated with other brain anomalies. However, MRI in two patients with absence of the septum pellucidum presented here, indicates that this anomaly may occur without associated anomalies. It may be one manifestation of a spectrum of developmental anomalies. One patient presented with schizophrenic psychosis; developmental disturbances in limbic areas are believed to be associated with schizophrenia. Agenesis of the septum pellucidum may indicate abnormal development of limbic structures and it may be associated with anomalies, such as cytoarchitectural disturbances of cortical layers, as yet undetectable by MRI.