Autoimmune encephalitis, epilepsy, autoimmune epilepsy
Conditions
Interventions
All patients receive 2 courses of IVIg
0.4 grams/kg/day for 5 days, starting on day 1 and day 22.
Sponsors
Erasmus Medical Center, Rotterdam, The Netherlands
Eligibility
Inclusion criteria
Inclusion criteria: - Age of 18 years and older. - Epilepsy, with at least one seizure per week at baseline. - Antibodies proven in serum and/or CSF in cell-based assay and/or ELISA and on immunohistochemistry. In case of anti-GAD antibodies, antibody titer with ELISA has to be >10,000 IU in serum or >100 IU in CSF.
Exclusion criteria
Exclusion criteria: - Age below 18 years. - Another identified cause of epilepsy (i.e. viral/bacterial encephalitis, stroke, tumor). - Severe encephalitis in which escalation of therapy (second-line immunotherapy, i.e. Rituximab or Cyclophosphamide) is expected within the study period (mainly anti-NMDAR encephalitis with mRS 5, at the ICU). - Use of immunotherapy < 3 months ago. - Use of monoclonal antibodies < 1 year ago. - Premorbid mRS =3. - Known hypersensitivity to Privigen or contraindication for Privigen, i.e. IgA deficiency. - Patient and/or legal representative is withholding informed consent. - Patient objects after initial informed consent. A potential subject who meets none of the above mentioned exclusion criteria, but meets one or more of the following exclusion criteria for TMS-EEG, will be excluded from TMS-EEG, but not from the rest of the study: - Incapacitation of the patient. - Cochlear implants, implanted neurostimulator or metal in the brain or skull. - Previous skull opening or trauma. - Cardiac pacemaker or intracardiac lines. - Pregnancy.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The proportion of patients with a seizure frequency reduction >50% and the proportion of patients that achieve full freedom of seizures in week 6, 7 and 8, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for the baseline frequency). | — |
Secondary
| Measure | Time frame |
|---|---|
| - Antibody levels pre- and post-treatment correlated to the seizure frequency, for all patients with autoimmune epilepsy and compared by subgroup. In addition, the amount of patients with antibody titer reduction >50% and the amount of patients that become antibody free, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for the baseline levels). - Clinical improvement: o Changes in the PNS neurological scale and FAB at 3, 6, 12 and 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for baseline scores). o Changes in the MOCA and QOLIE-31-P49,50 at 3, 6, 12 and 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for baseline scores). o Proportion of patients with a relapse within 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup at 18 weeks. Tertiary study parameters/endpoints - Clinical improvement: o Proportion of patients returning to a modified Rankin scale (mRS) of 0 or 1, at 3, 6, 12 and at 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for the baseline score). o Changes in the Extended Glasgow Outcome Scale (GOSE), Clinical Assessment Scale for Autoimmune Encephalitis (CASE) and AMC linear disability score (ALDS) at 3, 6, 12 and 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for baseline scores). o Changes in the Barthel index at 6, 12 and 18 weeks, for all patients with autoimmune epilepsy and compared by subgroup (adjusted for baseline score). o Seizure frequency reduction (absolute and relative numbers), for all patients with autoimmune epilepsy and compared by subgroup (adjusted for baseline frequency). o CGI-I scores at 5 days, and 3 weeks and 5 days, for all patients with autoimmune epilepsy and compared by subgroup. - Amount of patients with a normalization of the EEG, for all patients | — |
Contacts
Public ContactYvette Crijnen
Erasmus MC University Medical Center
Outcome results
None listed