CASPR2 Encephalitis Initial Symptoms (iniCASPR2)

Defining the Initial Clinical Symptoms and Characterizing Those Leading to the Discovery of CASPR2

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

Study Overview

• Status: Completed
• Conditions: Paraneoplastic Syndromes; CASPR2-Antibody; Encephalitis Autoimmune
• Intervention / Treatment: Other: CASPR2 encephalitis

• Study Type: Observational
• Enrollment (Actual): 48

Contacts and Locations

Study Locations

• France
  • Bron, France, 69500
    • Service de Neuro-Oncologie, Hôpital Neurologique Pierre Wertheimer

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients with a CASPR2 antibody-associated auto-immune encephalitis

Description

Inclusion Criteria:

• 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

Exclusion Criteria:

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

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort

Intervention / Treatment

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

Other: CASPR2 encephalitis; 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.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
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

Collaborators and Investigators

• Sponsor: Hospices Civils de Lyon
• Investigators: Principal Investigator: Jérôme HONNORAT, Pr, Service de neuro-oncologie, Hôpital neurologique

Study record dates

Study Major Dates

• Study Start (Actual): January 11, 2021
• Primary Completion (Actual): March 30, 2021
• Study Completion (Actual): January 20, 2022

Study Registration Dates

• First Submitted: November 27, 2020
• First Submitted That Met QC Criteria: November 27, 2020
• First Posted (Actual): December 4, 2020

Study Record Updates

• Last Update Posted (Actual): February 4, 2022
• Last Update Submitted That Met QC Criteria: February 3, 2022
• Last Verified: February 1, 2022

More Information

Terms related to this study

• Keywords: antibody ; auto-immune encephalitis ; paraneoplastic syndrome ; CASPR2
• Additional Relevant MeSH Terms: Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Neoplasms; Encephalitis; Paraneoplastic Syndromes
• Other Study ID Numbers: 69HCL20_0760

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No