Phase III Clinical Study of NPB-01 in Patients With Autoimmune Encephalitis

Inclusion Criteria

• < At 1st registration > Patients meeting the possible diagnostic criteria forautoimmune encephalitis
• < At 1st registration > Patients with a CASE score of 5 to 22 during the screeningperiod
• < At 1st registration > Patients with autoimmune encephalitis in progress (active andrequiring therapeutic intervention)
• < At 1st registration > IVIG therapy and steroid pulse therapy are considerednecessary by the investigator.
• < At 1st registration > Patients aged 15 years or older at the time of informedconsent
• < At 2nd registration > 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 ProbableAutoantibodies

• < At 2nd registration > CASE score of 5 to 22 on Day 8 of the previous treatmentperiod
• < At 2nd registration > Patients who have had an inadequate response to steroid pulsetherapy

Exclusion Criteria:

• < At 1st registration > Patients with strongly suspected infectious encephalitis
• < At 1st registration > Patients who received immunoglobulin preparations within 8weeks prior to informed consent
• < At 1st registration > Patients who received plasma exchange within 4 weeks prior toinformed consent
• < At 1st registration > Patients who received immunosuppressants (Rituximab,cyclophosphamide, etc.) within 4 weeks prior to informed consent
• < At 1st registration > Patients who have had tumor resection associated withautoimmune encephalitis within 4 weeks prior to informed consent
• < At 1st registration > Patients with a history of shock or hypersensitivity to theingredients of NPB-01
• < At 1st registration > Patients with known IgA deficiency
• < At 1st registration > Patients with renal disorder
• < At 1st registration > Patients with a current or previous history of cerebral orcardiovascular disorders (Asymptomatic cerebral infarction and myocardial infarctionthat occurred more than 5 years ago are not applicable.)
• < At 1st registration > Patients at high risk of thromboembolism
• < At 1st registration > Patients with haemolytic/blood loss anaemia
• < At 1st registration > Immunosuppressed/immunocompromised patients
• < At 1st registration > Patients with decreased cardiac function
• < At 1st registration > Pregnant, expected (desired or planned) pregnant, orbreastfeeding patients
• < At 1st registration > Use of prohibited medications or treatment in this study
• < At 1st registration > Patients who received investigational product in this study (re-enrollment prohibited)
• < At 1st registration > Patients who have received treatment with investigationalproduct other than this study within 4 months prior to informed consent
• < At 1st registration > Patients with a history of hypersensitivity tomethylprednisolone sodium succinate
• < At 1st registration > Patients who have a tumor associated with autoimmuneencephalitis and are considered to require resection during the study period.
• < At 1st registration > Patients receiving intravenous general anesthetics or sedativehypnotics
• < At 1st registration > Patients in coma
• < At 1st registration > Ventilated patients
• < At 1st registration > Patients who cannot undergo protocol-specifiedtests/assessments
• < At 1st registration > Other patients considered ineligible for the study by theinvestigator
• < At 2nd registration > Positive herpes simplex virus DNA qualitative test in thescreening period.
• < At 2nd registration > Serum creatinine ≥ 2 times the upper limit of normal duringthe screening period.
• < At 2nd registration > Total protein ≥ 9 g/dL during the screening period.
• < At 2nd registration > Patients with hematocrit ≥ 55% during the screening period
• < At 2nd registration > Patients who meet any of the exclusion criteria at the time offirst registration