Medium-term Effects of Treatments in Autoimmune Encephalitis

Hospices Civils de Lyon200 enrolled

Overview

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.

Study Type

OBSERVATIONAL

Enrollment

200

Conditions

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

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)

Eligibility

Sex: ALL

Medical Language ↔ Plain English

Inclusion Criteria: * 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. Exclusion Criteria: \- Refusal by the referring doctor to participate or refusal by the patient mentioned in the objection to the use of his/her clinical data.

Outcomes

Primary Outcomes

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.

Time frame: At baseline and 4 months after the initiation of therapy