Purpose: To study prospectively the evolution of visual attention in children with West syndrome to evaluate its development before the onset of spasms, its possible deterioration as a consequence of epileptic disorders, and its outcome at the age of 2 years, and the possible relation between the impairment of visual attention and cognitive development.
Methods: Infants with symptomatic West syndrome were examined before the onset of spasms and until age 24 +/- 2 months. Visual attention study (through a clinical observation and the fixation-shift test), cognitive assessment, and complete clinical examination including brain magnetic resonance imaging were performed.
Results: A maturation defect of fixation shift skills was generally observed in infants with West syndrome. In some cases, the impaired visual-attention abilities paralleled a cognitive deterioration, even months before the onset of spasms. During the acute stage of West syndrome, infants lost the previously acquired visual and cognitive abilities, with a typical fluctuation of arousal. Usually at 2 years, there was a persistent defective visual attention detected with the fixation-shift test.
Conclusions: The parallel defect of visual attention and of cognitive competencies is a constant finding in infants with West syndrome; these can precede the clinical onset of epileptic spasms. The severity and persistence of visual inattention might be explained by the age of visual maturation, corresponding to the usual onset of West syndrome.