Cortical visual impairment in children with infantile spasms.

BACKGROUND

Infantile spasms are often associated with cortical visual impairment.

METHOD

We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), 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 improvement 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 associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.