Table 1 Electroclinical characteristics of GGE patients with presentation of SE
From: Status epilepticus in patients with genetic generalized epilepsy: a case series study
Case (age, sex) | GGE syndrome | Age of onset in years, type of first seizure | Clinical presentation of SE | EEG characteristics (duration) | Possible cause of SE | ASMs before SE | Treatment at SE | Treatment after SE | Outcome and follow-up time |
|---|---|---|---|---|---|---|---|---|---|
1(8y, F) | EMA epilepsy | 6 years, EM | Eyelid myoclonus, consciousness impairment, occasional limb myoclonus, head jerk | 3–5 Hz generalized poly/spike-wave discharges (120 min) | No ASMs | None | MDZ | VPA (0.75 g/day) | Seizure-free (3years) |
2(10y, F) | EMA epilepsy | 10 years, GTCS | Increased heart rate, occasional eyelid myoclonus | Generalized slow waves admixed with spikes waves (twice, 15 and 19 min, respectively) | Ill-advised ASMs (OXC) | OXC (0.3 g/day) | None | LEV (1 g/day) | Seizure-free (0.25 year) |
3(19Y, F) | EMA epilepsy | 13 years, GTCS | Eyelid myoclonus, consciousness impairment, secondary GTCS | 3–5 Hz generalized poly/spike-wave discharges (46 min) | Ill-advised ASMs (OXC) | OXC (0.6 g/day) | MDZ | VPA (0.5 g/day) LEV (1.5 g/day) | Seizure-free (2 years) |
4(22y, F) | EMA epilepsy | 8 years, EMA | Eyelid myoclonus, consciousness impairment, occasional limb myoclonus, head jerk | Paroxysmal 2.5–3 Hz generalized polyspike/spike-wave discharges (60 min) | Insufficient ASMs (LTG) | LTG (0.025 g/day) | MDZ | LEV (1 g/day) TPM (0.15 g/day) | Infrequent seizures (1 year) |
5(24y, F) | EMA epilepsy | 15 years, GTCS | Eyelid myoclonus, slight limb myoclonus, consciousness impairment, secondary GTCS | Generalized polyspike/spike-wave discharges (180 min) | Ill-advised ASMs (CBZ) | VPA (1 g/day) CBZ (0.6 g/day) | None | VPA (1 g/day) TPM (0.2 g/day) | Seizure-free (2 years) |
6(12y, M) | JME | 8 years, MJ | Limb myoclonus, consciousness impairment, secondary GTCS | Irregular generalized slow waves admixed with polyspike/spike-wave discharges (35 min) | No ASMs | None | None | VPA (0.5 g/day) | Seizure-free (2 years) |
7(18y, F) | JME | 15 years, MJ | Limb myoclonus | Paroxysmal 3–4 Hz generalized polyspike/spike-wave discharges (180 min) | Insufficient ASMs (VPA) | VPA (1 g/day) | MDZ | VPA (1.5 g/d) | Seizure-free (6 years) |
8(31y, F) | JME | 17 years, GTCS | Limb myoclonus, secondary GTCS | Paroxysmal 3–4 Hz generalized polyspike/spike-wave discharges (30 min) | No ASMs | None | None | LTG (0.05 g/d) | Frequent seizures (5 years) |
9(7, M) | POMA epilepsy | 6 years, PMA | Perioral myoclonus, consciousness impairment | 2.5–3 Hz generalized spike-wave discharges (20 s) | No ASMs | None | None | VPA (0.5 g/d) | Seizure free (2 years) |