Table 4.
Epilepsy characteristics of the patients.
| Total | LGS with MRC I deficiency | LGS without MRC I deficiency | p-value | |
|---|---|---|---|---|
| (n = 68) | (n = 30) | (n = 38) | ||
| Seizure onset age, years, median (range) | 1 (0–7) | 1 (0–7) | 1 (0–5) | 0.630 |
| Seizure type, n (%) | ||||
| Generalized tonic and drop seizure | 64 (94.1) | 30 (100) | 38 (100) | 0.861 |
| Myoclonic | 15 (22.1) | 8 (26.7) | 7 (18.4) | |
| Atonic | 17 (25.0) | 7 (23.3) | 10 (26.3) | |
| Atypical absence | 4 (5.9) | 1 (3.3) | 3 (7.9) | |
| Focal | 16 (23.5) | 8 (26.7) | 8 (21.1) | |
| Status epilepticus, n (%) | 6 (8.8) | 3 (10.0) | 3 (7.9) | 0.544 |
| Number of anti-seizure medications ever used, median (range) | 4 (1–8) | 4.5 (2–8) | 3 (1–6) | 0.005 |
| History of diet therapy, n (%) | ||||
| Classic KD (4:1, 3:1) | 19 (27.9) | 10 (33.3) | 9 (23.7) | 0.300 |
| MAD | 11 (16.2) | 4 (13.3) | 7 (18.4) | |
| LGIT (low glycemic index) | 2 (2.9) | 0 | 2 (5.3) | |
| History of epilepsy surgery, n (%) | ||||
| Corpus callosotomy | 3 (4.4) | 1 (3.3) | 2 (5.3) | 0.459 |
| VNS insertion | 1 (1.5) | 0 | 1 (2.6) | |
| Resective surgery | 3 (4.4) | 0 | 3 (7.9) | |
| Change in seizure frequency, n (%) | ||||
| Reduction rate of all seizures at the 1-year follow-up | ||||
| 0–25% | 22 (32.4) | 9 (30.0) | 13 (34.2) | 0.704 |
| 25–50% | 10 (14.7) | 6 (20.0) | 4 (10.5) | |
| 50–75% | 6 (8.8) | 3 (10.0) | 3 (7.9) | |
| 75–100% | 30 (44.1) | 12 (40.0) | 18 (47.4) | |
| Reduction rate of all seizures at the last follow-up | ||||
| 0–25% | 12 (17.6) | 7 (23.3) | 5 (13.2) | 0.481 |
| 25–50% | 15 (22.1) | 7 (23.3) | 8 (21.1) | |
| 50–75% | 5 (7.4) | 3 (10.0) | 2 (5.3) | |
| 75–100% | 36 (52.9) | 13 (43.3) | 23 (60.5) | |
| Reduction rate of generalized tonic and drop seizures at the 1-year follow-up | ||||
| 0–25% | 31 (45.6) | 20 (66.7) | 11 (28.9) | < 0.001 |
| 25–50% | 11 (16.2) | 7 (23.3) | 4 (10.5) | |
| 50–75% | 10 (14.7) | 1 (3.3) | 9 (23.7) | |
| 75–100% | 16 (23.5) | 2 (6.7) | 14 (36.8) | |
| Reduction rate of generalized tonic and drop seizures at the last follow-up | ||||
| 0–25% | 22 (32.4) | 18 (60.0) | 4 (10.5) | < 0.001 |
| 25–50% | 12 (17.6) | 9 (30.0) | 3 (7.9) | |
| 50–75% | 12 (17.6) | 1 (3.3) | 11 (28.9) | |
| 75–100% | 22 (32.4) | 2 (6.7) | 20 (52.6) | |
| Genetic test results, n (%) | (N=42) | 0.475 | ||
| Pathogenic variant | 11 (26.2) | 7 (16.7) | 4 (9.5) | |
| Negative finding | 31 (73.8) | 13 (31.0) | 18 (42.9) | |
LGS, Lennox–Gastaut syndrome; MRC I, mitochondrial respiratory chain complex I; KD, ketogenic diet; MAD, modified Atkins diet; LGIT, low glycemic index treatment; VNS, vagus nerve stimulation.