POSTERIOR-FOSSA ABNORMALITIES IN CHILDREN WITH INFANTILE SPASMS

In order to explore possible pathophysiologic involvement of the brain stem in infantile spasms, we retrospectively compared clinical and el ectroencephalographic (EEG) features of 14 children with infantile spa sms who had gross posterior fossa abnormalities on neuroimaging studie s with 84 children with infantile spasms who had either normal neuroim aging (n = 19) or supratentorial abnormalities (n = 65). Children with posterior fossa abnormalities had lower mean initial and follow-up de velopmental quotients compared to those with normal imaging or suprate ntorial abnormalities alone. Age of onset of infantile spasms, latency to treatment, response to steroids, and follow-up EEG pattern were no t significantly different among the three groups. Six children (6%) ha d Dandy-Walker cysts, an association rarely reported with infantile sp asms. We conclude that the presence of posterior fossa abnormalities i n patients with infantile spasms portends a relatively poor developmen tal outcome.