Purpose: To investigate the significance of cortical pathology of tonic spa
sms in patients with tuberous sclerosis.
Methods: The subjects were 38 patients with epilepsy associated with tubero
us sclerosis. We analyzed ictal EEGs of tonic spasms and partial seizures b
y means of video-EEG monitoring for a total of 763 tonic spasms in 20 patie
nts and 107 partial seizures in 15 patients. We also investigated the relat
ion between partial seizures and magnetic resonance imaging (MRI) findings
of these patients.
Results: Ictal EEG patterns of tonic spasms were divided into generalized a
nd focal patterns. Thirteen patients had only generalized patterns, whereas
seven had both patterns. In five patients who had focal ictal patterns of
tonic spasms and partial seizures, the location of focal patterns correspon
ded with the location of onset of partial seizures. Focal discharges were s
een immediately before, after, and in the middle of tonic spasms in series
in 13 patients. The location of focal discharges also corresponded with the
location of the onset of partial seizures in 10 of the 13 patients. Regard
ing partial seizures, four patients had multiple active epileptogenic foci
during the same period, and two others had shifting epileptogenic foci with
increasing age.
Conclusions: These findings indicate that cortical pathology plays an impor
tant role in the occurrence not only of partial seizures but also of tonic
spasms in patients with tuberous sclerosis.