SEPTO-OPTIC DYSPLASIA - THE CLINICAL INSIGNIFICANCE OF AN ABSENT SEPTUM-PELLUCIDUM

Neurodevelopmental assessments were performed on seven patients with o ptic nerve hypoplasia and absence of the septum pellucidum on MRI. The evaluation included neurological status, language development, neurop sychological functioning, and behavioral and emotional adjustment. Six of the seven were found to have normal cognitive development, intact neurological status, normal language development and age-appropriate b ehavior. Abnormal findings included early poor motor coordination, whi ch was felt to be closely related to decreased visual acuity, as well as subtle visual attentional problems which occurred even in patients who had normal vision in one eye. Congenital absence of the septum pel lucidum was not associated with significant intellectual, behavioral o r neurological deficits in the majority of these patients.