TABLE 2.
Demographic data of patients.
| Patient | BA (pt 1) | NP (pt 2) | DD (pt 3) | EM (pt 4) |
|---|---|---|---|---|
| Age of onset | 4 m | 4.5 m | 7 m | 6 m |
| Age | 9 y | 14 y | 3y 2m | 8 y |
| First seizure | ||||
| Type | Clonic unilat. sn | GTC | GTC | Clonic unilat. dx |
| Duration | 30 min | 25 min | 20 min | 20 min |
| Fever association | Yes | Yes | Yes | Yes |
| Vaccine association | Yes | No | No | No |
| Interictal EEG | nl | nl | nl | nl |
| PMD | nl | nl | nl | nl |
| AED (after 1st seizure) | CBZ | VPA | VPA | VPA |
| Other reported seizures’types | ||||
| Myoclonic attacks | Yes | Yes | Yes | Yes |
| Myoclonic status | No | No | Yes | Yes |
| GTCS | Yes | Yes | Yes | Yes |
| Unilateral motor | Yes | Yes | No | Yes |
| Atypical absences | Yes | No | No | Yes |
| Partial seizures | No | Yes | No | Yes |
| Tonic (age of onset-end) | 2 y 10 m - ? | No | y (3–5y) | 4 y - ? |
| Fever sensitivity | +++ | +++ | ++ | ++ |
| Seizure in cluster | No | Yes | Yes | Yes |
| Treatment (onset-end) | ||||
| VPA | 14 m - now | 5.5m - | 11m - | 6 m - now |
| FB | 6 m – 2 y 7 m | No | No | Yes |
| CLB | 4 y - now | 5y - | 2.5y - | 1 y - now |
| CLZ | 14 m – 3 y | No | No | No |
| NZP | No | No | No | Yes |
| STP | 2 y 7 m – 4 y 8 m | 7y - | 2.5y - | 1 y - now |
| TPM | No | 12–13y | 7y - | 5 y – 6 y |
| ETS | 2 y 10 m - 4 y 8 m | No | No | No |
| CBZ | 5 m – 6 m | No | No | Yes |
| VGB | No | No | No | No |
| PHT | No | No | No | ? - 1 y 9 m |
| LVC | No | 13y- | No | ? - 7 y |
| LTG | No | 5–5.5y | No | No |
| PRM | No | No | No | Yes |
| ZNS | No | No | No | 7 y – 7 y 9 m |
| Hydrocortisone | 4 y 8 m - ? | No | No | 5 y 8 m – 6 y |
| Ketogenic diet | No | No | No | Yes |
| Vagal stimulator | No | No | No | No |
| Language | Simple S | short sentences | isolated words | No |
| Psychomotor delay | Moderate | moderate | severe | Moderate- Severe |
| SCN1A mutations | c.680 T>C | C3880-1G>T intron 19 | C4582-1G>T | IVS8+1C>T |