Table 1.
Clinical, genetic, neuropathological and electrophysiological features
| Patient | SE1 | SE2 | SE3 |
|---|---|---|---|
| Neuropathology | FCDIIa | FCDIIa | FCDIIa |
| Genetic findings | AKT3 p.E17K somatic | AKT3 p.E17K somatic | DEPDC5 2-hit |
| Sex | Female | Male | Female |
| Age at seizure onset | 15d | 4y | 6m |
| Age at latest follow-up | 3y | 12y | 7y |
| Number of surgeries | 2 | 3 | 1 |
| Seizure types | Infantile spasms, focal seizures | Focal seizures, loss of consciousness | Focal seizures, auras |
| Seizure frequency | Daily | Daily | Daily |
| Antiseizure medications | TPM, VGB, OXC | CBZ, CLB, VPA | LTG, CLB, VPA, LEV |
| 3 T MRI | Diffuse frontal lobe FCD | Postcentral and superior parietal FCD | Superior frontal lobe and precentral gyrus blurring |
| Scalp EEG (ictal) | L frontal rapid discharge | R fronto-central rapid discharge | R fronto-central rapid discharge |
| Stereo-EEG (ictal) | Rhythmic activation/rapid discharge in supramarginal and postcentral gyri; rapid discharge in superior frontal gyrus and motor operculum | Rapid discharges in pre- and postcentral and premotor cortex | Discharges in precentral gyrus |
| EZN | Pre- and postcentral gyri, premotor frontal cortex, insula, and motor operculum | Mesial central-SMA and postcentral gyrus | Precentral gyrus |
| Electrodes in MRI lesion | AS, FA, FS, IA, MS, OM, PA, PI, PS | FA, FS, MS, PA | LP, FA, FS |
| Electrodes in EZN | FA, PI, PS, IA OM | FS, MS, PA | LP, FS |
| Electrodes in PZN | AS, FS, IA, OM, OP, MS, PA, PP, | PA, MS, FS, OM, PA | FA, OM, PS, LP, FS |
| Electrodes in NIN | TA, TS, TP | AS, BL, BM, CA, FI, LS, OM, OP, PI, PP, | BL, BM, FB, FI, FM |
| Thermocoagulated contacts | AS3-10, FA7-16, IA2-18, MS1-7, OM1-4, PA4-6, PI1-11, PS1-7 | FS1-5, MS1-5, PA1-5, PA15-18 | None |
| Follow-up post-RFTC | NA | Electroclinical improvement for 2m | NA |
| SEEG-guided surgery | Yes | No | No |
d, days; m, months, y, years; FU, follow-up; EZN, epileptogenic zone network; PZN, propagation zone network; NIN, non-involved network; RFTC, radiofrequency thermocoagulation; SMA, supplementary motor area; TPM, topiramate, VGB, vigabatrin, OXC, oxcarbazepine, CBZ, carbamazepine; VPA, sodium valproate; CLB, clobazam; LTG, lamotrigine; LEV, levetiracetam. Following a medication change to felbamate post-SEEG, seizure frequency was reduced in patient SE3 (1-year follow-up period).