A Multicenter Randomized, Controlled, Double-blinded Trial to Evaluate Efficacy and Safety of Bortezomib in Patients With Severe Autoimmune Encephalitis

ZKSJ0120

Autoimmune Encephalitis is a disorder of the central nervous system caused by bodily substances, called antibodies. Antibodies normally help the body to prevent infections. However, in this disorder, the antibodies turn against the body itself and especially against cells in the brain and disturb the normal brain function. They are therefore called autoantibodies. There is no specific therapy for patients with autoimmune encephalitis so far. At the moment, the symptoms are treated with approved medications such as cortisone and immunotherapies also used in oncology. These therapies are unspecified and aim to reduce the number of autoantibodies and to contain the autoimmune process. In this trial we aim to test a new therapy option: in this therapy the body cells producing autoantibodies will be specifically targeted by a substance called bortezomib. The trial addresses patients with severe autoimmune encephalitis. The aim of the trial is to evaluate the efficacy and safety of bortezomib in patients with severe autoimmune encephalitis.

2020-05-13

2025-11-30

2026-04-30

Recruiting

Early phase I

50

Inclusion Criteria: * Clinically diagnosed severe autoimmune encephalitis (defined as mRS ≥ 3) with autoantibodies to neuronal surface proteins in cerebrospinal fluid and / or serum * Pretreatment with rituximab * Age ≥18 years * signed informed consent * Women of childbearing potential (up to 2 years after menopause): negative pregnancy test Exclusion Criteria: * pregnancy/breast-feeding * acute infiltrative pulmonary and pericardial disease * malignant tumor under current chemotherapy * Simultaneous participation in another intervention study * Previous participation in this study * Known hypersensitivity to an ingredient of the investigational product * Continued therapy with glucocorticoids / rituximab during the study duration (last dose must be administered before the first dose of the investigational product)

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2024-03-12