RecruitingPhase 2NCT03993262

Trial to Evaluate Efficacy and Safety of Bortezomib in Patients With Severe Autoimmune Encephalitis

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


Sponsor

Jena University Hospital

Enrollment

50 participants

Start Date

May 13, 2020

Study Type

INTERVENTIONAL

Conditions

Summary

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.


Eligibility

Min Age: 18 Years

Inclusion Criteria5

  • 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 Criteria7

  • 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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Interventions

DRUGBortezomib

1 to 3 cycles Bortezomib with 1,3mg/m2 body surface s.c. + 20mg dexamethasone p.o. on days 1, 4, 8 and 11 (= 1 cycle)

DRUGPlacebo

1 to 3 cycles placebo (NaCl solution) s.c. + 20mg dexamethasone p.o. on days 1, 4, 8 and 11 (= 1 cycle)


Locations(17)

Ludwig-Maximilians-Universität München, Klinikum Großhadern

München, Bavaria, Germany

Universitätsklinikum Würzburg

Würzburg, Bavaria, Germany

Universitätsklinikum Jena, Sektion Translationale Neuroimmunologie, Klinik für Neurologie

Jena, Germany, Germany

Medizinische Hochschule Hannover

Hanover, Niedersachen, Germany

Charité - Universitätsmedizin Berlin, Klinik für Neurologie mit Experimenteller Neurologie

Berlin, Germany

Ruhr-Universität Bochum, St. Josef Hospital, Klinik für Neurologie

Bochum, Germany

University Hospital Düsseldorf, Clinic for Neurology

Düsseldorf, Germany

Universitätsklinikum Erlangen, Neurologische Klinik

Erlangen, Germany

Universitätsklinikum Essen (AöR), Klinik für Neurologie

Essen, Germany

University Hospital Frankfurt (Main), Clinic for Neurology

Frankfurt, Germany

Universitätsmedizin Göttingen Georg-August-Universität, Klinik für Neurologie

Göttingen, Germany

Universitätsmedizin Greifswald, Klinik und Poliklinik für Neurologie

Greifswald, Germany

Klinik für Neurologie UKSH, Campus Kiel

Kiel, Germany

Universitätsklinikum Leipzig, Klinik und Poliklinik für Neurologie

Leipzig, Germany

Universitätsmedizin Mainz, Klinik und Poliklinik für Neurologie

Mainz, Germany

Universitätsklinikum Münster Klinik für Neurologie

Münster, Germany

Universitätsklinikum Ulm, Klinik für Neurologie Neurologische Ambulanz

Ulm, Germany

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NCT03993262


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