S. Francione et al., Epilepsy surgery in tuberous sclerosis., DRUG-RESISTANT SEVERE PARTIAL EPILEPSY IN CHILDREN: DIAGNOSTIC STRATEGIES AND SURGICAL TREATMENTS, 1998, pp. 278-295
Tuberous sclerosis (TS) is a multisystemic disease frequently associated wi
th a severe drug-resistant partial epilepsy. Nevertheless, the frequent pol
ymorphism of ictal clinical features, the extent of the interictal EEG abno
rmalities and the disseminated cerebral lesions probably explain why a surg
ical treatment is rarely decided with the aim to suppress the seizures.
Five patients with TS (including 2 "formes frustes") operated on at Grenobl
e Hospital are reported. The age at intervention was comprised between 24 a
nd 72 months. In all the patients, seizures started precociously (1-42 mont
hs) and were very frequent (2-15 fits a day), clinical semiology has evolve
d from the onset of seizures and only one patient presented one kind of sei
zure at intervention Interictal scalp EEG showed slow waves and spikes wide
ly extended in four patients and unilobar in one. Neuroimaging (CT-scan and
MRI) showed calcified lesions in all the patients, multiple in three cases
and single in two. A video-EEG monitoring was always performed while a ste
reo-EEG investigation was judged necessary in two cases. After the interven
tion (lesionectomy associated with a corticectomy irt thr-ee cases, single
lesionectomy in the remaining two), all the patients but one are seizure-fr
ee (follow-up 34-76 months). These data suggest that, in spite of multiple
lesions and widespread EEG abnormalities, the surgical treatment of partial
epilepsy in TS may prove to be very usefull in well-selected cases.