Autoimmune Encephalitis
Conditions
Interventions
- Experimental: NPB-01: Intravenous immunoglobulin
- 400 mg(8mL) /kg/d (max 1g/d) , 5 consecutive days, IV administration
- Active Comparator: NPB-01-ME: Methylprednisolone sodium succinate
-
Sponsors
Ota Mamoru
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) Patients aged 15 years or older at the time of informed consent 2) Patients who meet any of the following (1) to (6): (1) Definite diagnostic criteria for autoimmune limbic encephalitis (2) MRI evidence of demyelination (probable autoimmune encephalitis) (3) Probabilistic diagnostic criteria for anti-NMDAR encephalitis (4) Probabilistic diagnostic criteria for Bickerstaff brainstem encephalitis (5) Probabilistic diagnostic criteria for Hashimoto's encephalopathy (6) Diagnostic Criteria for Autoimmune Encephalitis with Negative but Probable Autoantibodies 3) CASE score of 5 to 22 4) Patients who have had an inadequate response to steroid pulse therapy
Exclusion criteria
Exclusion criteria: 1) Patients with strongly suspected infectious encephalitis 2) Patients who received immunoglobulin preparations within 8 weeks prior to informed consent 3) Patients who received plasma exchange within 4 weeks prior to informed consent 4) Patients who received immunosuppressants (Rituximab, cyclophosphamide, etc.) within 4 weeks prior to informed consent 5) Patients who have had tumor resection associated with autoimmune encephalitis within 4 weeks prior to informed consent 6) Patients with a history of shock or hypersensitivity to the ingredients of NPB-01 7) Patients with known IgA deficiency 8) Patients with renal disorder 9) Patients with a current or previous history of cerebral or cardiovascular disorders (Asymptomatic cerebral infarction and myocardial infarction that occurred more than 5 years ago are not applicable.) 10) Patients at high risk of thromboembolism 11) Patients with haemolytic/blood loss anaemia 12) Immunosuppressed/immunocompromised patients 13) Patients with decreased cardiac function 14) Pregnant, expected (desired or planned) pregnant, or breastfeeding patients 15) Use of prohibited medications or treatment in this study 16) Patients who received investigational product in this study (re-enrollment prohibited) 17) Patients who have received treatment with investigational product other than this study within 4 months prior to informed consent 18) Patients with a history of hypersensitivity to methylprednisolone sodium succinate 19) Patients who have a tumor associated with autoimmune encephalitis and are considered to require resection during the study period. 20) Patients receiving intravenous general anesthetics or sedative hypnotics 21) Patients in coma 22) Ventilated patients 23) Patients who cannot undergo protocol-specified tests/assessments 24) Other patients considered ineligible for the study by the investigator or subinvestigator 25) Positive herpes simplex virus DNA qualitative test in the screening period. 26) Serum creatinine 2 times or more the upper limit of normal during the screening period. 27) Total protein 9 g/dL or more during the screening period. 28) Patients with hematocrit 55% or more during the screening period
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1.Percentage of Responders Based on Clinical Assessment Scale in Autoimmune Encephalitis (CASE) Score at Week 4 of the Post-Treatment Observation Period Timeframe: Week 4 A responder was defined as a participant whose CASE (Clinical Assessment Scale in Autoimmune Encephalitis) score at Week 4 of the post-treatment observation period after study treatment with investigational product improved by 40% or more compared to the pre-treatment. The CASE score is an assessment index created to evaluate the symptoms of autoimmune encephalitis and consists of 9 items (seizures, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness). Each item is assigned a value of up to 3 points, and the total score ranges from 0 to 27. A higher score indicates greater severity of autoimmune encephalitis. | — |
Secondary
| Measure | Time frame |
|---|---|
| 1.Change From Baseline in CASE Score Timeframe: Pre-treatment (Baseline), Week 1, 2, 3, 4, 6, 8, 12 2.Number of Participants in Each Categorized CASE Score Timeframe: Pre-treatment (Baseline), Week 1, 2, 3, 4, 6, 8, 12 Number of participants in each category of CASE score (0 - 4: excellent, 5 - 9: moderate, 10 - 27: poor) at each time point was reported. 3. Time to CASE Score Improvement Timeframe: Up to 12 weeks from the start of treatment period Time from the start of treatment with study drug until CASE score improvement was reported. CASE score improvement was defined as decrease to 4 or less of the CASE score. 4.Change From Baseline in Modified Rankin Scale (mRS) Score Timeframe: Pre-treatment (Baseline), Week 1, 2, 3, 4, 6, 8, 12 The mRS score is a general prognostic scale for stroke patients and consists of 7 scales (0: Completely asymptomatic to 6: Death) and the total score ranges from 0 to 6. A higher score indicates greater severity of stroke. 5.Change from Baseline in Glasgow Coma Scale (GCS) Score Timeframe: Pre-treatment (Baseline), Week 1, 2, 3, 4, 6, 8, 12 The GCS score was used as an assessment index for level of consciousness. GCS quantifies three components: eye opening, verbal response, and motor response, and evaluates consciousness levels based on the total score. Total score ranges from 0 to 15. Lower scores indicate greater severity, and a score of 8 or less is considered severe. 6.Change From Baseline in Mini-Mental State Examination-Japanese (MMSE-J) Score Timeframe: Pre-treatment (Baseline), Week 4, 8, 12 MMSE-J is the Japanese version of the MMSE (Mini-Mental State Examination), used dementia screening test. MMSE-J consists of questions related to 11 categories measuring cognitive function: orientation to time, orientation to place, registration, attention and calculation, recall, naming, repetition, comprehension, reading, writing, and drawing. Scores range from 0 to 30 with lower values indicating greater impairment. 7.Change Fro | — |
Contacts
Public ContactResearch and Development division Nihon Pharmaceutical Co., Ltd.
Nihon Pharmaceutical Co., Ltd.
Outcome results
None listed