Medium-term Effects of Treatments in Autoimmune Encephalitis

NCT07133113 | Recruiting

• 1 other identifier: 24-5346
• Study Type: observational
• Target: 200
• Locations: 1 country
• Sites: 1

Brief Summary

Autoimmune encephalitides are severe neurological disorders requiring urgent treatment, even though there is no standard guideline by lack of empirical evidence. Commonly used treatments are divided into so-called first-line (steroids, intravenous immunoglobulins, plasma exchanges) and second-line (rituximab, cyclophosphamide, tocilizumab, others), and may be used in association or sequentially. There is no standard practice, and initial treatment protocol may consist in first-line alone, first-line with rituximab, or first-line with dual immunosuppression (rituximab and cyclophosphamide). Absence of clear response to initial treatment in the first 4 to 6 weeks may indicate undertreatment and is generally followed by treatment escalation, mostly to dual immunosuppression. However, as the frequency of non-responders to initial treatment is unknown, it is still unclear whether dual immunosuppression should be offered to all patients from inception.

Conditions

NMDAR Autoimmune Encephalitis; LGI1 Antibody Associated Encephalitis; CASPR2-Antibody; IgLON5; GAD65; GFAP

Keywords

Paraneoplastic neurological disorder; autoimmune encephalitis

Outcome Measures

Primary Outcomes (1)

• Failure of the initial treatment protocol

  Failure of the initial treatment protocol, reflected by the decision to escalate treatment between V1 and V2. Treatment escalation is defined as the addition of one or more second-line treatments more than 30 days after the start of the initial treatment.

  At baseline and 4 months after the initiation of therapy

Study Arms (6)

group 1 : Patients with NMDAR encephalitis

Patients with untreated anti-NMDAR encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

group 2 : Patients with GAD encephalitis

Patients with untreated anti-GAD encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

group 3 : Patients with LGI1 encephalitis

Patients with untreated anti-LGI1 encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

group 4 : Patients with IgLON5 encephalitis

Patients with untreated anti-IgLON5 encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

group 5 : Patients with GFAP encephalitis

Patients with untreated anti-GFAP encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

group 6 : Patients with CASPR2 encephalitis

Patients with untreated anti-CASPR2 encephalitis or with a decision to treat within the previous 30 days

Other: We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression).

Interventions

We aim to assess the clinical response to the treatment initiation protocols most commonly used in autoimmune encephalitis (first-line, first-line with rituximab, dual immunosuppression). OTHER

The patients will be identified via the French Nationwide Multidisciplinary Team Meetings for Autoimmune encephalitis, which are conducted on a bi-monthly basis. All patients with newly diagnosed NMDAR, LGI1, CASPR2, IgLON5, GFAP or GAD65 encephalitis and treated for less than 8 weeks will be included. Treatment protocols at the initiation of therapy will be stratified into: 1) first-line only; 2) rituximab without cyclophosphamide; 3) rituximab combined with cyclophosphamide; 4) others. The referral clinicians will be contacted by email and the data will be collected using standardized questionnaires, which will be sent at baseline (visit 1, V1) and 4 months after the initiation of therapy (visit 2, V2). The questionnaires will be structured into 7 sections: * 1 Demographics * 2 Symptoms * 3 Cognitive screening tests (MMSE, MoCA, and/or others) * 4 Level of dependence (ADL, I-ADL, mRS, CASE) and impact on social life * 5 Diagnostic tests (brain MRI, brain PET, CSF, and EEG findings)

group 1 : Patients with NMDAR encephalitis; group 2 : Patients with GAD encephalitis; group 3 : Patients with LGI1 encephalitis; group 4 : Patients with IgLON5 encephalitis; group 5 : Patients with GFAP encephalitis; group 6 : Patients with CASPR2 encephalitis

Eligibility Criteria

• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

All Patients with autoimmune encephalitis defined anti-GAD NMDAR, LGI1, CASPR2, IgLON5 or GFAP and untreated within the previous 30 days

You may qualify if:

• Adult or child patient with encephalitis defined as anti-GAD, NMDAR, LGI1, CASPR2, IgLON5 or GFAP
• Untreated or with a decision to treat within the previous 30 days.

You may not qualify if:

• \- Refusal by the referring doctor to participate or refusal by the patient mentioned in the objection to the use of his/her clinical data.

Sponsors & Collaborators

• Hospices Civils de Lyon: lead

Study Sites (1)

Hospices Civil de Lyon

Bron, 69677, France

RECRUITING

Biospecimen

Retention: SAMPLES WITH DNA

serum, DNA, CSF, PBMC

MeSH Terms

Conditions

Autoimmune Diseases of the Nervous System

Interventions

Rituximab

Condition Hierarchy (Ancestors)

Nervous System Diseases; Autoimmune Diseases; Immune System Diseases

Intervention Hierarchy (Ancestors)

Antibodies, Monoclonal, Murine-Derived; Antibodies, Monoclonal; Antibodies; Immunoglobulins; Immunoproteins; Blood Proteins; Proteins; Amino Acids, Peptides, and Proteins; Serum Globulins; Globulins

Central Study Contacts

Bastien Pr JOUBERT

CONTACT

04 78 86 17 89; bastien.joubert@chu-lyon.fr

Géraldine PICARD

CONTACT

04.72.35.58.42; geraldine.picard@chu-lyon.fr

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: August 13, 2025
• First Posted: August 20, 2025
• Study Start: September 1, 2024
• Primary Completion: September 1, 2024
• Study Completion (Estimated): March 1, 2027
• Last Updated: August 20, 2025

Record last verified: 2025-03

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)