Table 2.
Systematic review of anti-IL-1 agents in epilepsy.
| References | Disease | N, (age) | Study design | Intervention | Timing | Efficacy | Safety |
|---|---|---|---|---|---|---|---|
| Sa et al. (105) | FIRES | 2 Pt 1: 1.9 years Pt 2. 2.5 years |
Case Report | Pt 1 Anakinra 5 mg/Kg/day s.c for 14 days |
Start: Day 43 | From Day 51 seizures decreased in frequency and on Day 60 these stopped. After 3 weeks new onset of seizures (2–5/month) |
No adverse effects |
| Pt 2 Anakinra 10 mg/Kg/day s.c for 90 days |
Start: day 22 Discontinued after 3 months of treatment |
No improvement | No adverse effects | ||||
| Yang et al. (106) | FIRES | 1 (6 years old) |
Case Report | Anakinka 100 mg s.c. twice daily for 1 year | Start: Day 28 | Resolution of seizures after 4 days. Stop ketogenic diet after 9 months. Follow-up: 1 seizure/month |
No adverse effects |
| Kern-Smith et al. (107) | NORSE | 1 (5 years old) |
Case Report | Anakinra for 13 days (posology not specified) | Start: Day 12 Stop: Day 24 |
Stop midazolam infusion, without return of electrographic status epilepticus, after 2 days | No adverse effects |
| Dilena et al. (108) | FIRES | 1 (10 years old) |
Case Report | Anankinra 2.5 mg/kg/day (100 mg) s.c and, 3 days after, 2.5 mg/kg twice daily | Start: after 18 months from diagnosis Stop: 7 months after |
Full seizure control after 3 days | No adverse effects |
| Jyonouchi and Geng (109) | ESES | 1 (6 years old) |
Case Report | Anakinra 100 mg/day s.c. | Start: 25 months after diagnosis | Despite the improvement of behavioral symptoms, ESES pattern persisted. | No adverse effects |
| Lai et al. (110) | FIRES | 25, (5–11 years old) | Retrospective | Anakinra 3–5 mg/kg/day (initial dose) Anakinra 4–9 mg/kg/day (final dose) |
Start: 20 days after the onset of FIRES Stop: 86 days after |
Earlier anakinra initiation after seizure onset was associated with shorter duration of mechanical ventilation, and ICU and hospital LOS. Amongst children with available seizure frequency data, 11/15 (73.3%) exhibited> 50% seizure reduction at 1 week of anakinra treatment. 3/25 (12%) died |
3/25 (12%) developed DRESS 2/25 (8%) developed cytopenia 10/25 (40%) developed infections (1 discontinued anakinra for infections) |
| Westbrook et al. (111) | FIRES | 1 (21 years old) |
Case Report | Anakinra 100 mg 3 times daily s.c (Initial dose) Anakinra 100 mg twice daily s.c (after 10 days) Anakinra 100 mg once daily s.c. (after 25 days) |
Start: 32 days after the diagnosis of FIRES Stop: 1 year after at which time discontinuation will be discussed |
Full seizure control after 24 h | No adverse effects |
| Kenney- Jung et al. (112) | FIRES | 1 (32 months old) |
Case Report | Anakinra 5 mg/kg/twice daily s.c. Anakinra 5 mg/kg/twice daily s.c. |
2 cycles: Day 6–23 Day 54-ongoing |
Improved seizure control in both cycles (from 5.8 to 1.3 seizure/day in the first cycle; from 8 to 0.17 seizure/day in the second). Twelve months after initial presentation, the patient experiences rare focal seizures. |
Development of DRESS (day 22, followed by discontinuation) |
| DeSena et al. (113) | DRE | 1 (14 years old) |
Case Report | Anakinra 100 mg daily Anakinra 100 mg twice daily Canakinumab 300 mg every 4 weeks |
Start: 2 years Stop: After 4 weeks After: 2 months |
Rapid ~80% reduction in seizure frequency (from 4 to 15/day to 4/week). No clinically evident seizures. Improvements in her fatigue, general malaise, quality of life, and academic performance. Long periods of being seizure-free, currently averaging one seizure per several months. |
No adverse effects |
| Stredny et al. (114) | FIRES | 1 (6 years old) |
Case Report | Anakinra 20 mg/kg daily | From day 6 to 20 of hospitalization | No clinical response | No adverse effects |
| Mochol et al. (115) | RE | 1 (43 years old) |
Case Report | Anakinra 100 mg daily sc | 26 years after disease presentation. 1st cycle: 2 months 2nd cycle: 7 months |
Complete seizure control after 1 week of treatment. Relapse after 2 weeks of withdrawal 2nd cycle: full seizure control after 10 days 13 months seizure-free |
Pneumonia |
| Choi et al. (116) | SRSE in AE | 1 (38 years old) |
Case Report | Anakinra 100 mg daily s.c | From week 12 Duration: 12 days |
Resolution of status epilepticus Recovery in communication and walking) |
No adverse effects |
AE, autoimmune encephalitis; DRE, drug-resistant epilepsy; DRESS, Drug rash with eosinophilia and systemic symptoms; ESES, Encephalopathy with electrical status epilepticus in sleep; FIRES, Febrile infection-related epilepsy syndrome; NORSE, New-onset refractory status epilepticus; RE, Rasmussen encephalitis; SRSE, super-refractory status epilepticus.