To determine a management strategy for the
epilepsy in children with bilateral cortical malformations, clinical data of 23 patients (age, 3-23 years, M:F=7:16) were retrospectively reviewed. Among these patients, 15 were bedridden and 16 were profoundly retarded and could not even smile. The patients were categorized into the following five groups based on the findings of neuroimaging, seizure types, and electroencephalographic patterns. Group 1: Diffuse cortical malformation with epileptic
spasms and secondarily generalized
tonic seizures, group 2: diffuse cortical malformation with erratic twitches, group 3: bilaterally extended but not diffuse cortical malformations, group 4: bilateral
polymicrogyria with persistent epileptic
spasms (
Aicardi syndrome), and group 5: bilateral cortical malformation with
drop attacks (
subcortical band heterotopia and
congenital bilateral perisylvian syndrome). Eleven patients suffered from
infantile spasms;
adrenocorticotropic hormone was effective in group 1 but ineffective in group 4. Treatment of
tonic seizures in groups 1-3 and erratic twitching in group 3 with
phenobarbital,
zonisamide and
potassium bromide was beneficial. Epileptic
spasms and
tonic seizures were prominent in group 4 and were refractory to medical treatment, except that
zonisamide,
clobazam, and a
ketogenic diet were partially or transiently effective. Complex partial and astatic/
atonic seizures in group 5 were refractory to medications other than that
carbamazepine and
clobazam provided limited benefits. Total callosotomy resulted in better seizure control for three patients in group 5, and
functional hemispherectomy was effective for one patient in group 4. These results provide the basis for the appropriate choice of medical and surgical treatment for managing bilateral, widespread cortical malformations.