Table 5.
Systematic review of anti-CD20 agents in epilepsy.
| References | Disease | N, (age) | Study design | Intervention | Timing | Efficacy | Safety |
|---|---|---|---|---|---|---|---|
| Cheli et al. (134) | Anti-LGI1 encephalitis with DRE | 1 (54 years old) |
Case Report | Rituximab 1,000 mg/day IV, 2 doses 15 days apart then one single dose after 6 months. | Start: 7 weeks after the onset of seizures. Stop: 9 weeks after the onset of seizures. |
No further seizures occurred after the treatment. The neuropsychological evaluation resulted within normal range. After 6 months, the patient experienced a cognitive relapse that resolved after the administration of a single dose of Rituximab. | No adverse effects |
| Kurukumbi et al. (133) | Patient 1: anti-NMDR encephalitis with RSE Patient 2: anti- LGI1 encephalitis with DRE Patient 3: N-type anti-VGCC encephalitis with RSE |
3 Pt 1: 32 years Pt 2: 72 years Pt 3: 19 years |
Case Series | Pt 1: Rituximab 375 mg/m2/day IV weekly for 4 weeks, then rituximab 1,000 mg IV every 6 months Pt 2: Rituximab 1,000 mg IV every 6 months Pt 3: Rituximab 1,000 mg IV every 6 months |
Pt 1, start: 27 days after the onset of seizures. Pt 2, start: 12 months after diagnosis Pt 3, start: 5 days after the onset of seizures. |
Pt 1: resolution of seizures and behavioral disorders, with a return to baseline cognition and personality Pt 2: electrographic and clinical seizure freedom with return of premorbid cognitive function Pt 3: abrogation of seizures and return to baseline functioning |
No adverse effects |
| Sansevere et al. (132) | RE | 1 (11 years old) |
Case Report | Rituximab 375 mg/m2 weekly for 4 weeks | Start: 5 days after diagnosis Stop: 33 days after diagnosis |
No clinical response. Functional hemispherectomy with right hemisphere deafferentation was performed 3 months after the final dose of rituximab | No adverse effects |
| Schneider et al. (131) | Anti-NMDAR encephalitis with RSE | 1 (22 years old) |
Case Report | Rituximab 500 mg IV, followed by a second dose after 6 months and a third after 16 months | Start: not specified Stop: 16 months after the first administration |
Complete remission of epileptic seizures and psychotic symptoms | No adverse effects |
| Jun et al. (124) | NORSE* | 6 [median 36 years (22–61)] |
Prospective | Rituximab 375 mg/m2/day IV weekly | Not specified | Persistence of SE despite the treatment. Patients eventually received Tocilizumab | No adverse effects |
| El Tawil et al. (130) | RE with DRE | 1 (61 years old) |
Case Report | Rituximab (posology not specified) | Start: 10 years after diagnosis Stop: not specified |
Clear and sustained improvement in seizure frequency and severity and patient's disabilities | No adverse effects |
| Timarova et al. (129) | RE with RSE | 1 (32 years old) |
Case Report | Rituximab 375 mg/m2/day IV weekly (two cycles 22 months apart) | Start: not specified Stop: 22 months after the first administration |
Reduction of epileptic seizures with residual 2–3 partial seizures per week. Worsening 18 months after (partial seizures rose to 6 per day). After the second cycle, persistence of one partial seizure per day. | No adverse effects |
| Byun et al. (128) | AE** | 12 [median 32 years (18–68)] |
Prospective study | Rituximab 375 mg/m2/day IV weekly for 4 weeks. The treatment was then repeated every month. | Start: 3–9 weeks after first immunotherapy cycle (with steroid or IVIg). | Remission of seizures in 8/12 patients at 6 months; seizures reduction > 50% in 1/12 patient; no clinical change in 3/12 patients. | Infusion-related reactions (headache, dizziness, chest discomfort) (n = 2); rash with pruritus 24 h after the infusion (n = 1). This patient discontinued the treatment. |
AE, autoimmune encephalitis; DRE, drug-resistant epilepsy; IV, intravenous; IVIg, intravenous immunoglobulins; LGI1, leucine rich glioma inactivated 1; NMDAR, N-methyl-D-aspartate receptor; NORSE, New-onset refractory status epilepticus; RE, Rasmussen encephalitis; SRSE, super-refractory status epilepticus; VGCC, voltage-gated calcium channel.
*
One patient suffered from anti-NMDAR encephalitis, in the other cases the disease was cryptogenic.
**
AE in these patients was due to anti-NMDAR (n = 8), anti-LGI1 (n = 3), and anti-Ma2/Ta (n = 1).