Hemimegalencephaly and neurofibromatosis

Abstract

Hemimegalencephaly, which previously has been associated with a poor clinical course characterized by intractable seizures and severe encephalopathy, was found without these conditions in two children with neurofibromatosis. These children showed relatively similar and favourable prognostic features: no presence of seizures before one month, seizures controlled or absent, no focal neurological signs and peculiar EEG findings. In our opinion the absence of heterotopias and hamartomas can be related with less severe outcome.