phase iii clinical study of npb-01 in patients with autoimmune encephalitis

Conditions: Autoimmune Encephalitis

Summary: to compare the efficacy and safety of npb-01 in patients with autoimmune encephalitis refractory to steroid pulse therapy using steroid pulse therapy as a control.


Eligibility criteria:
inclusion criteria * \ patients meeting the possible diagnostic criteria for autoimmune encephalitis * \ patients with a case score of 5 to 22 during the screening period * \ patients with autoimmune encephalitis in progress (active and requiring therapeutic intervention) * \ ivig therapy and steroid pulse therapy are considered necessary by the investigator. * \ patients aged 15 years or older at the time of informed consent * \ 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 * \ case score of 5 to 22 on day 8 of the previous treatment period * \ patients who have had an inadequate response to steroid pulse therapy exclusion criteria: * \ patients with strongly suspected infectious encephalitis * \ patients who received immunoglobulin preparations within 8 weeks prior to informed consent * \ patients who received plasma exchange within 4 weeks prior to informed consent * \ patients who received immunosuppressants (rituximab, cyclophosphamide, etc.) within 4 weeks prior to informed consent * \ patients who have had tumor resection associated with autoimmune encephalitis within 4 weeks prior to informed consent * \ patients with a history of shock or hypersensitivity to the ingredients of npb-01 * \ patients with known iga deficiency * \ patients with renal disorder * \ 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.) * \ patients at high risk of thromboembolism * \ patients with haemolytic/blood loss anaemia * \ immunosuppressed/immunocompromised patients * \ patients with decreased cardiac function * \ pregnant, expected (desired or planned) pregnant, or breastfeeding patients * \ use of prohibited medications or treatment in this study * \ patients who received investigational product in this study (re-enrollment prohibited) * \ patients who have received treatment with investigational product other than this study within 4 months prior to informed consent * \ patients with a history of hypersensitivity to methylprednisolone sodium succinate * \ patients who have a tumor associated with autoimmune encephalitis and are considered to require resection during the study period. * \ patients receiving intravenous general anesthetics or sedative hypnotics * \ patients in coma * \ ventilated patients * \ patients who cannot undergo protocol-specified tests/assessments * \ other patients considered ineligible for the study by the investigator * \ positive herpes simplex virus dna qualitative test in the screening period. * \ serum creatinine ≥ 2 times the upper limit of normal during the screening period. * \ total protein ≥ 9 g/dl during the screening period. * \ patients with hematocrit ≥ 55% during the screening period * \ patients who meet any of the exclusion criteria at the time of first registration