Two distinctive electroencephalographic abnormalities, very high ampli
tude rhythmic activity or prominent fast activity, have been described
in children with extensive cortical dysplasia. Cases with cortical dy
splasia identified on computerised tomography or magnetic resonance im
aging, or a characteristic EEG were selected. One hundred and forty el
ectroencephalograms from 94 cases were reviewed and related to the ima
ging findings. An EEG with very high amplitude rhythmic activity was f
ound to have high specificity for severe cortical dysplasia but low se
nsitivity (< 50% cases). Abnormal fast activity was not specific and w
as seen with very diverse pathologies. The EEG features of most cases
with localised cortical dysplasia were very variable. The EEG could be
normal even when the cortical dysplasia was extensive. These differen
ces emphasise the need for both detailed neuroimaging and neurophysiol
ogical investigations when assessing children with neurological proble
ms.