[Neuro-ophthalmological and radiological signs of Aicardi syndrome]

Described in the paper are the results of ophthalmologic, ultrasound and radiological examinations of 3 girls with the Aicardi syndrome. It was for the first time, that an expansion of the optic-nerve disk excavation was described in patients with the above syndrome; such expansion was conditioned by trans-synaptic neuron degeneration induced by lesions to optic radiation in view of prenatal of encephaloplastic processes. The changing brain white substance as projected by optic radiation was detected in all children by computer and magnetic resonance tomography. Besides, other anomalies of the optic nerve (bindweed syndrome, coloboma) and of the retina are elucidated. Finally, the pathogenesis and differential diagnostics of the Aicardi syndrome are under discussion.