Cortical visual impairment in children with infantile spasms

Background: Infantile spasms are often associated with cortical visual impa irment. Method:We report a series of 10 patients with infantile spasms, hyp sarrhythmic electroencephalograms, and developmental delay, tie, West syndr ome), who presented with severe visual inattention despite a normal ocular examination. Results: At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of impr ovement was observed in the others, their visual function remained abnormal . All patients had moderate or severe mental retardation. Conclusions: The clinical features of West syndrome are reviewed, and the cause of the assoc iated cortical visual impairment is discussed. Cortical visual impairment w ith infantile spasms is an important association, which pediatric ophthalmo logists should recognize.