Malformations of cortical development (MCD) are an important aetiology
of localization-related epilepsy. Previous MRT and [C-11]flumazenil P
ET studies have demonstrated widespread structural and neuroreceptor a
bnormalities beyond the region of MCD that is visually apparent on MRI
. We investigated the ability of brain regions affected by MCD to part
icipate in normal cognitive and motor tasks and compared the responses
seen in such patients with those in normal subjects. We studied five
patients known to have MCD affecting the occipital region and seven no
rmal subjects using H-2 O-15 PET whilst they were performing a visual
attention task. We also studied five right-handed patients known to ha
ve MCD affecting the left frontal lobe and seven right-handed normal s
ubjects, using H-2 O-15 PET whilst they were performing a motor learni
ng task with the right hand. The patient and normal control data were
examined using statistical parametric mapping to determine the ability
of the brain region affected by MCD to participate in the task and al
so to detect evidence for atypical organization of cortical function i
n association with the MCD, Eight of the ten patients with MCD showed
significant alteration of relative regional cerebral blood flow during
the task compared with 'rest' in the affected brain region. These reg
ions included focally dysgenetic cortex, the cortex lining schizenceph
alic clefts, heterotopic bands, subependymal grey matter heterotopia,
and the cortex overlying band and subependymal heterotopia. In additio
n there was a significant alteration in the overall activation pattern
in five patients compared with the normal control groups; in all five
patients this atypical organization involved regions of cortex that a
ppeared entirely normal on MRI, We conclude that regions of MCD may pa
rticipate in normal cognitive functions but widespread cortical atypic
al organization may be seen. These findings have implications for surg
ical planning in any such patients.