TABLE 1.
Characteristics of patients with new onset refractory SE based on etiology and presence of fever
| Characteristic | All cases, n = 46 | NORSE with unknown etiology, n = 40 | NORSE with known etiology, n = 6a | ||
|---|---|---|---|---|---|
| Fever ≤24 h prior, FSE, n = 19 | Fever >24 h prior, FIRES, n = 16 | No fever, n = 5 | |||
| Age, years | 2.4 [1.2–8.6] | 1.2 [.9–1.7] | 5.3 [2.4–8.9] | 10.2 [2.3–10.2] | 6.3 [3.2–11.0] |
| Sex, male | 21 (46) | 10 (52) | 8 (50) | 3 (60) | 0 (0) |
| Prodromal symptoms | 28 (61) | 6 (31) | 16 (100) | 1 (20) | 5 (83) |
| Fever still present at SE onset | 33 (75) | 19 (100) | 12 (75) | 0 (0) | 3 (50) |
| SE duration, h, n = 44 | 24 [7–128] | 10 [4–33] | 684 [103.5–1158] | 12 [8.2–17.2] | 60 [30–126] |
| Duration of ICU stay, days | 3.5 [2–24] | 2 [1–3] | 49 [24–64] | 1.5 [1–2] | 6 [4–8] |
| Epileptiform discharges, n = 42 | 20 (48) | 3/16 (19) | 11/15 (73) | 2/5 (40) | 4/6 (67) |
| Seizures | 13 | 2 | 8 | 0 | 3 |
| Periodic discharges | 12 | 1 | 10 | 0 | 1 |
| Sporadic epileptiform discharges | 12 | 2 | 5 | 2 | 3 |
| Abnormal MRI, n = 37 | 20 (46) | 1/12 (8) | 13/16 (69) | 2/5 (40) | 4 (67) |
| T2/FLAIR hyperintensity | 18 | 0 | 12 | 2 | 3 |
| DWI hyperintensity | 12 | 0 | 10 | 0 | 1 |
| Abnormal CSF, n = 41 | 14 (39) | 1/16 (6) | 10/16 (63) | 0/3 (0) | 3 (50) |
| Elevated protein level | 12 | 0 | 8 | 0 | 3 |
| Elevated white cell count | 11 | 1 | 8 | 0 | 2 |
| Non‐BZD ASM | 2 [2–2] | 2 [1–2] | 3 [2–3] | 2 [1–2] | 2 [2–2] |
| Continuous infusion | |||||
| n (%) | 25 (54) | 8 (42) | 13 (81) | 2 (40) | 2 (33) |
| Median [IQR] | 1 [0–1] | 0 [0–1] | 2 [1–3] | 1 [0–1] | 0 [0–1] |
| Ketogenic diet | 9 (19) | 0 | 9 (56) | 0 (0) | 0 (0) |
| Immunotherapies | 12 (26) | 0 | 11 (69) | 1 (20) | 0 (0) |
| IV steroids | 11 | 10 | 1 | ||
| IVIG | 9 | 9 | 0 | ||
| Plasma exchange | 3 | 3 | 0 | ||
| Complications, any | 20 (43) | 3 (16) | 13 (81) | 1 (20) | 3 (50) |
| Good outcome | 28 (61) | 17 (89) | 4 (25) | 4 (80) | 3 (50) |
Data are presented as n (%) or median [IQR].
Abbreviations: ASM, antiseizure medication; BZD, benzodiazepine; CSF, cerebrospinal fluid; DWI, diffusion‐weighted imaging; FIRES, febrile infection‐related epilepsy syndrome; FLAIR, fluid‐attenuated inversion recovery; FSE, febrile SE; ICU, intensive care unit; IQR, interquartile range; IV, intravenous; IVIG, intravenous immunoglobulin; MRI, magnetic resonance imaging; NORSE, new onset refractory SE; SE, status epilepticus.
a
NORSE with known etiology; the etiology was initially unknown but found after extensive investigations.