CASPR2 Encephalitis Initial Symptoms

NCT04653571 | Completed

• 1 other identifier: 69HCL20_0760
• Study Type: observational
• Target: 48
• Locations: 1 country
• Sites: 1

Brief Summary

Auto-immune encephalitides involve auto-antibodies targeting the central nervous system, and particularly the synapse and its structure, such as protein CASPR2. CASPR2 antibody-associated auto-immune encephalitides lead to an inflammation of the limbic system and generate focal temporal seizures and cognitive impairment. Most patients are initially hospitalized because of the temporal seizures (Joubert et al., JAMA Neurology 2016). However, many already show at that time cognitive impairment, which has failed to elicit the appropriate investigations, therefore delaying the diagnosis. The study will hence investigate precisely the initial, sometimes neglected, clinical symptoms and those leading to the diagnosis, in the cohort of patients suffering from a CASPR2 antibody-associated encephalitis, from the French reference center on paraneoplastic neurological diseases and autoimmune encephalitis. This way, the study aims to delineate the symptoms that should trigger suspicions of a CASPR2 antibody-associated encephalitis

Conditions

CASPR2-Antibody; Encephalitis Autoimmune; Paraneoplastic Syndromes

Keywords

antibody ; auto-immune encephalitis ; paraneoplastic syndrome ; CASPR2

Outcome Measures

Primary Outcomes (1)

• Initial cognitive impairment or clinical presentation of first epileptic seizures.

  The retained information will depend on the very first declared symptom: if the patient first showed focal seizures, we will precise the clinical presentation of this seizure. On the other hand, if the patient first showed cognitive symptoms, we will best define this impairment.

  8 months

Study Arms (1)

CASPR2 encephalitis

Cohort of patients with a CASPR2 antibody-associated auto-immune encephalitis.

Other: CASPR2 encephalitis

Interventions

CASPR2 encephalitis OTHER

Defining the initial clinical symptoms and those leading to the diagnosis of CASPR2 antibody associated encephalitis. For that, we will investigate the initial cognitive status, the initial neuropsychological investigations the clinical presentation of the first seizures, and the prodromes assessed by patients at the beginning of their disease. We will also look at the initial EEG records, in order to acknowledge whether the initial cognitive symptoms may be due to unseen seizures.

CASPR2 encephalitis

Eligibility Criteria

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

Study Population

Patients with a CASPR2 antibody-associated auto-immune encephalitis

You may qualify if:

• Positive CASPR2-Ab in serum and/or CSF tested by immunohistochemistry on mouse brain slices and confirmed by cell-based assay (CBA) of HEK293 cells expressing CASPR2.
• Diagnosis and follow-up in France
• From 18 to unlimited age

You may not qualify if:

• Patients CASPR2-IgG negative in serum and CSF
• Foreign follow-up

Sponsors & Collaborators

• Hospices Civils de Lyon: lead

Study Sites (1)

Service de Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer

Bron, 69500, France

Location

MeSH Terms

Conditions

Autoimmune Diseases of the Nervous System; Paraneoplastic Syndromes; Cortical Dysplasia-Focal Epilepsy Syndrome

Condition Hierarchy (Ancestors)

Nervous System Diseases; Autoimmune Diseases; Immune System Diseases; Neoplasms

Study Officials

• Jérôme HONNORAT, Pr

  Service de neuro-oncologie, Hôpital neurologique

  PRINCIPAL INVESTIGATOR

Study Design

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

Study Record Dates

• First Submitted: November 27, 2020
• First Posted: December 4, 2020
• Study Start: January 11, 2021
• Primary Completion: March 30, 2021
• Study Completion: January 20, 2022
• Last Updated: February 4, 2022

Record last verified: 2022-02

Data Sharing

• IPD Sharing: Will not share

Locations

FR (1)